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Review

A Comprehensive Literature Review on Diagnostic Strategies and Clinical Outcome of Intraoral Angiosarcoma and Kaposi Sarcoma

by
Primali Rukmal Jayasooriya
1,
Hiruni Ashcharya Wijerathna Weerasinghe
1,
Liyanaarachchige Anushan Hiranya Jayasinghe
2,
Prasangi Madubhashini Peiris
3,
Wijeyapala Abeyasinghe Mudiyanselage Udari Lakshika Abeyasinghe
1 and
Ruwan Duminda Jayasinghe
3,*
1
Department of Oral Pathology, Faculty of Dental Sciences, University of Peradeniya, Peradeniya 20400, Sri Lanka
2
Ministry of Health, Suwasiripaya, No. 385, Rev. Baddegama Wimalawansa Thero Mawatha, Colombo 01000, Sri Lanka
3
Department of Oral Medicine & Periodontology, University of Peradeniya, Peradeniya 20400, Sri Lanka
*
Author to whom correspondence should be addressed.
J. Vasc. Dis. 2024, 3(3), 306-318; https://doi.org/10.3390/jvd3030024
Submission received: 14 June 2024 / Revised: 13 July 2024 / Accepted: 20 August 2024 / Published: 30 August 2024
(This article belongs to the Section Peripheral Vascular Diseases)
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Abstract

:
This review analyzes the clinicopathological features, diagnostic challenges, and clinical outcomes of 60 intraoral angiosarcoma (InO-AS) and 20 intraoral Kaposi sarcoma (InO-KS) cases. These malignancies primarily affect adults, with mean ages of 52.3 years for InO-AS and 44 years for InO-KS, and are rare in children. Both show a male predilection, with InO-KS strongly linked to HIV infection. Metastatic InO-AS typically appears smaller and is located in the mandibular or maxillary gingiva. Most InO-KS cases occur in HIV-positive individuals, often in nodular form. Histological differentiation of InO-AS from poorly differentiated carcinoma and spindle cell carcinoma requires a comprehensive panel of immunohistochemical markers such as CK, CD31, and CD34, while HHV-8 antibody and CD34 help diagnose InO-KS. Treatment for InO-AS involves surgery with radiotherapy and/or chemotherapy, while InO-KS management may include antiretroviral therapy for AIDS patients. InO-AS is aggressive, with over half of patients dying from the disease, whereas InO-KS generally has a less severe course. Despite their rarity, both InO-AS and InO-KS behave similarly to their extraoral counterparts. A key limitation noted in this review is the inconsistent histopathological reporting of AS, particularly regarding histopathological grade, which complicates the assessment and comparison of treatment outcomes.

1. Introduction

Intraoral malignant vascular tumors, particularly angiosarcomas and Kaposi sarcomas, are exceptionally rare compared to benign and reactive lesions of vascular origin. This literature review concentrates specifically on these two malignant vascular tumors, excluding atypical vascular tumors and hemangioendotheliomas. Due to its scarcity and diverse histopathological presentations, diagnosing angiosarcomas poses a significant challenge as they often resemble poorly differentiated carcinomas, pleomorphic sarcomas, and various other sarcomas with spindle cell differentiation. Conversely, Kaposi sarcoma presents a spectrum of histopathological changes, mimicking benign and reactive lesions like dermatitis in its initial patch stage to sarcomas exhibiting spindle cell differentiation in its nodular stage [1].
Angiosarcoma is a very rare malignant tumor originating from cells exhibiting endothelial differentiation and accounts for less than 1% of all soft tissue sarcomas [1]. Considering its anatomic distribution, approximately half of the tumors occur in cutaneous tissues followed by parenchymal organs with a predilection to breast and heart. The third and fourth most common locations are deep soft tissues and bone, respectively [2]. Due to clinical implications, angiosarcomas are divided in to five clinical subgroups, namely, (1) Primary cutaneous angiosarcoma (unassociated with lymphedema or radiation), (2) Lymphedema associated with angiosarcoma, (3) Post irradiation angiosarcoma, (4) Angiosarcoma of deep soft tissues, and (5) Angiosarcoma of parenchymal organs [1].
In the era of personalized medicine, it is worthwhile to describe the molecular genetics of angiosarcomas known to date. Angiosarcomas are characterized by over-expression of genes implicated in different stages of angiogenesis, including genes for vascular-specific receptor tyrosine kinase. Gene expression profiles are different for radiation-induced and non-radiation-induced angiosarcomas, namely the former lesions being characterized by over-expression of LYN and PRKθ and the latter lesions being characterized by over-expression of FLT1 and AKT3. High-level MYC amplification has been described for post radiation and lymphedema-associated angiosarcoma [1].
Kaposi sarcoma is a rare vascular tumor (multifocal angio-proliferative disease) which usually occurs in skin and mucosal surfaces also with the potential of involvement of viscera. It is a soft tissue tumor that can be seen in several distinct populations. Moritz Kaposi, an Austro-Hungarian dermatologist, was the first to report five cases of Kaposi sarcoma in 1872 under the term “idiopathic multiple hemorrhagic sarcoma” [3,4,5]. Human Herpes virus-8 (HHV-8) and immunosuppression have been identified as major risk factors for Kaposi sarcoma [6,7]. It can be divided into four clinico-epidemiological subtypes as follows: (1) Classic form, (2) Endemic/African form, (3) HIV-related form and (4) Iatrogenic form.
The classic form of Kaposi sarcoma is seen among Mediterranean and Eastern European descent and there is a higher risk for secondary malignancies in this form. The endemic form has shown a greater predilection for the pediatric population and presents with generalized lymph node involvement. It has shown a strong relationship with HHV-8. The HIV-related form is considered as an AIDS defining illness [8]. It is the second most common tumor in HIV patients, with CD4 counts less than 200 cells/mm3. Up to 30% of patients who are not on HAART (high-activity antiretroviral therapy) will develop Kaposi sarcoma [7]. Among the reported cases of oral Kaposi sarcoma, the HIV-related form was the commonest presentation. The iatrogenic form occurs in immunosuppressed patients such as transplant recipients and patients on long-term steroid therapy, other immunosuppressive agents or antitumor necrosis factor therapy [7].
Oral Kaposi sarcoma clinically presents as a violaceous pink to purple color lesion exhibiting its vascular nature. The mucocutaneous lesions progress through patch, plaque and nodular clinical stages. Lesions can become ulcerative and painful during their clinical presentation. Depending on the stage of the Kaposi sarcoma, it can mimic oral pathologies such as nevi, pyogenic granuloma, bacillary angiomatosis, hemangioma, angiosarcoma or melanoma. Thorough clinical and laboratory evaluation is important in correct diagnosis of Kaposi sarcoma [6].
In spite of oral mucosa or jaw bones being uncommon sites for occurrence of angiosarcoma and Kaposi sarcoma, the literature reveals the existence of angiosarcoma as primary [9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25,26,27,28,29,30,31,32,33,34,35,36,37,38,39,40,41,42,43] or metastatic tumor [24,44,45,46,47,48,49,50] and Kaposi sarcomas [4,51,52,53,54,55,56,57,58,59,60,61,62,63,64,65] within the oral cavity. Therefore, the present review aims to highlight demographic features and the diagnostic and management challenges of primary and metastatic angiosarcomas and Kaposi sarcoma of the oral cavity based on 60 cases of primary angiosarcomas (which includes three unpublished cases diagnosed by the present authors in Table 1) and 14 cases of metastatic angiosarcomas (Table 2) and 20 cases of Kaposi sarcomas (Table 3) published in the literature. By compiling the available literature on primary and metastatic intraoral angiosarcomas (InO-As) and Kaposi sarcomas, we hope to enhance understanding among clinicians and researchers, ultimately facilitating improved diagnosis and management strategies for these challenging entities.

2. Methodology

A PubMed and a Google Scholar search was conducted in April 2024 using the key terms “Kasposi sarcoma of the oral cavity” and “Angiosarcoma of the oral cavity”. The search strategy included case reports published from 1949 to 2024. Only papers published in the English literature were included in this review. Three unpublished cases of intraoral angiosarcomas reported at the Department of Oral Pathology, Faculty of Dental Sciences, University of Perdaeniya, Sri Lanka were also included. The information retrieved from the above search was used in the compilation of the present article.

3. Results and Discussion

3.1. Angiosarcoma

The literature review revealed that the average age at presentation of primary oral cavity angiosarcoma was 52.3 years within a wide age range spanning from 6 to 86 years [9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25,26,27,28,29,30,31,32,33,34,35,36,37,38,39,40,41,42,43]. Interestingly, 12% occurred in children, while the remaining patients were adults (88%). Approximately 37% of the patients were elderly and in the seventh to ninth decades of life (Table 2). The age range and percentage of pediatric primary oral cavity angiosarcomas identified in the present literature review are similar to the findings reported by Di Battista et al., though the average age at presentation was a decade younger than compared to the above study [26]. Further compared to Head and Neck angiosarcomas with a mean age of 73 years at presentation, there is a tendency for the primary oral cavity angiosarcomas to present at a younger age [66]. However, this discrepancy could also be attributed to the fact that a relatively higher percentage of pediatric primary angiosarcomas was identified in the oral cavity as per the present literature review—12% (Table 2) vs. 0.48% in the article by Lee et al. [66]. In contrast to primary oral cavity angiosarcomas, 14 metastatic oral cavity angiosarcomas were only identified in adults with an average age of 63.8 years at presentation and within an age range of 34–83 years (Table 2). This age distribution is similar to that of Fanburg-Smith et al. reported for a series of metastatic oral cavity angiosarcomas [24].
The present literature review revealed almost equal gender distribution, with a male to female ratio of 1.3:1 for primary oral cavity angiosarcomas, which is supported by the findings of Di Battitsa et al. [26]. In contrast, metastatic oral cavity angiosarcomas presented with a male predilection (3.67:1), which is supported by Fanburg-Smith et al. [24]. With respect to site distribution, primary angiosarcomas were found to occur in all oral mucosal sites as well as being involved in both jaw bones (Table 2). Thirty-five tumors (61.4%) affected purely soft tissues [10,11,12,16,17,18,19,20,21,22,23,24,26,34,39,42,43], while twenty-two (38.6%) had either maxillary or mandibular bone involvement as well [9,10,13,14,25,31,32,33,35,36,37,38,40,41]. The commonest site of occurrence of primary angiosarcomas was tongue (21/60 = 35%), while maxillary and mandibular gingiva in combination accounted for 41.6% (25/60) of the tumors [10,12,17,18,19,20,21,22,23,24,26,27,67]. However, when considering these two locations separately, maxillary gingiva with or without bone involvement accounted for 10% (6/60) of tumors [9,10,31,43], whilst mandibular gingiva with or without bone involvement accounted for 31.6% of tumors (19/60) [10,13,14,15,25,29,30,32,33,35,36,37,38,39,40,42]. In contrast, all metastatic tumors occurred in either mandibular or maxillary gingiva [26,44,45,46,47,48,50,68] and followed a similar pattern to those reported by Fanburg-Smith et al. [24]. Though, an exact reason could not be identified for the predilection shown by metastatic angiosarcomas for the gingival tissue, it can be hypothesized that gingival tissue being a common site of inflammation may inadvertently have a good blood supply, allowing for a higher rate of metastasis at this site, or may contain favorable soil expressing significant molecules that have an affinity for neoplastic endothelial cells.
Generally, primary angiosarcomas of the oral cavity present as reddish to purple-colored expansile growths with or without ulceration [10,11,14,24,26,40]. When the lesions have been large at presentation, clinicians have suspected malignant lesions. However, smaller lesions have carried clinical differential diagnoses of benign or reactive lesions such as pyogenic granuloma, hemangiomas or epulides [27] and, therefore, it is important to recognize that some angiosarcomas may present as clinically indolent lesions. The size was mentioned only in 30 primary lesions [9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25,27,29,30,31] and 6 metastatic lesions [44,46,47,50]. Initially, the size was categorized considering 5 cm as the cutoff mark, as the literature revealed prognostic relevance in terms of size [66,69,70]. Out of the 30 lesions, 46.6% [10,11,13,14,15,18,19,20,21,22,27,30] of the primary lesions were larger than 5 cm, while 53.3% [9,10,12,14,16,23,24,29,67] of the lesions were smaller than 5 cm. All metastatic tumors were smaller than 5 cm [44,46,47,50]. Awareness of the primary tumor or effects of primary tumors elsewhere in the body may contribute to patients seeking treatment when lesions are quite small and result in metastatic tumors being smaller than some primary tumors at diagnosis.
Traditionally, sarcomas are classified considering 5 cm as the cutoff mark. However, the eighth edition of the American Joint Committee on Cancer (AJCC) Staging Manual recommends that since Head and Neck sarcomas are smaller than sarcomas that occur at other sites, T staging should be followed similar to other Head and Neck tumors considering 2 and 4 cm tumor cut-off points, as well as T4 classification based on invasion of adjacent structures [71]. Using the eighth edition of the AJCC Staging Manual as a guideline, an attempt was made to classify oral cavity angiosarcomas published in the literature. Accordingly, all tumors with bone involvement in the absence of the size were considered as T4 tumors. T stage could be determined for 36 tumors [9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,27,29,30,31,32,33,35,36,37,38,40,41,67], out of which a majority of 58.3% were T4 tumors followed by 19.5% of T3 tumors. T1 and T2 tumors comprised 13.8% and 8.4% of the sample, respectively. The literature review revealed information on TNM stage in only nine primary angiosarcomas [10,12,18,19,20,21,22,24], out of which nodal metastasis was identified in 44.4% (4/9) of angiosarcomas. Information related to distant metastasis was available in 37 patients [9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25,26,28,30,31,32,36,38], out of which 11 patients (29.7%) presented with distant metastasis [10,14,17,19,20,22,23,36,38]. The commonest site for distant metastasis of angiosarcoma was lung (5/11) followed by bone (3/11) and skin (2/11) [10,14,17,19,20,22,23,38]. However, as it was not possible to determine the cutoff point considered in deciding the T stage in published cases, no attempt was made to compare the outcome based on TNM stage.
When analyzing the literature, oral cavity angiosarcomas were found to comprise three types of cells, namely epithelioid cells, spindle cells and anaplastic cells in isolation or in various combinations [2,3,4,5,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25,26,27,28,29,30,31,32,33,34,35,36,37,38,39,40,41,42,43]. In addition, the literature also described a vasoformative pattern where irregular anatomizing vascular channels were present lined by atypical cells [26]. Therefore, histopathologically, oral cavity angiosarcoma is a diagnostically challenging entity due to cellular and morphological diversities exhibited both within a single tumor and between tumors. Di Battista, in their review, has described epithelioid and spindle cell angiosarcomas, with the epithelioid variant being slightly more common than the spindle cell variant [27]. The definitive diagnosis of angiosarcoma can only be achieved with immunohistochemistry-based confirmation of the vascular phenotype with vascular markers such as CD 31, CD 34, von Willebrand factor (vWF), FLI1 and ERG. In the absence of newer antibodies such as such as FLI1 and ERG, which are highly sensitive and specific markers for angiosarcoma, it is recommended to use both CD31 and CD34, which will allow the confirmation of almost 95% of angiosarcomas, even poorly differentiated anaplastic tumors [1]. While immunohistochemistry can provide valuable information by detecting specific markers associated with vascular differentiation, it is essential to interpret these findings within the broader context of thorough histopathological analysis, as both malignant and benign tumors are positive for vascular markers. This involves considering various factors such as cellular morphology, growth pattern, and clinical history to accurately differentiate between benign and malignant vascular tumors.
Histopathological differential diagnoses of epithelioid angiosarcoma include epithelioid hemangioendothelioma (EHE), squamous cell carcinoma and epithelioid sarcoma. Unlike epithelioid angiosarcoma, EHE is a tumor that has an indolent behavior and commonly occurs in younger patients. Its characteristic histopathology of cords and nests of polygonal endothelial cells with intracellular lumina and WWTR1–CAMTA1 fusion, which is specific for EHE, are features that could be used to differentiate the two tumors as both are positive for vascular markers such as CD 31 and CD 34 as well as epithelial markers in 25–40% of cases. Squamous cell carcinomas and epithelioid sarcomas could be differentiated from epithelioid angiosarcoma due to lack of expression of vascular markers CD 31 in the former tumors [1,27]. In the oral cavity, spindle cell angiosarcomas should especially be differentiated from spindle cell carcinoma, in addition to other sarcomas with spindle cell differentiation (Figure 1). Spindle cell carcinomas could be differentiated from angiosarcoma by the presence of a characteristic carcinoma component and its expression of epithelial markers in the absence of vascular marker expression [1,27].
Angiosarcomas are histologically graded based on a three-tier grading as low, intermediate and high grade [14,17,24,25,26,29,30]. However, this grading system is not uniform, as sometimes the tumors were either graded as G1—well differentiated; G2—moderately differentiated; G3—poorly differentiated; and G4—undifferentiated [10,11,23,66], or diagnosed only as epithelioid or spindle cell pattern. In the present literature review, 27 primary angiosarcomas [10,11,14,17,24,25,26,29,30] and 5 metastatic angiosarcomas [26,49] carried a grade. Accordingly, 40% of the primary tumors were high grade and 30% each were low and intermediate grade. All metastatic oral cavity angiosarcomas were high grade [26,49]. Fanburg-Smith et al. report that grade is not a reliable prognostic marker for angiosarcomas of the oral cavity, and this could be a reason why grade is not consistently included in the literature [24]. Similarly, when head and neck angiosarcomas are considered, Lee et al. reveals age, tumor size, and disease extent as determinants of survival [66]. They also showed that when all other factors were controlled, lower histologic grade and surgery did not improve the risk of death.
The treatment was only mentioned for 37 primary [10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25,27,28,29,30,31,32,33,34,35,36,37,41,42,67] and 7 metastatic [44,45,46,47] angiosarcoma of the oral cavity. The majority (20/37; 33.3%) of primary angiosarcomas were treated by only surgery. A further 11 patients were treated with surgery and adjuvant radiotherapy or chemotherapy. The literature also confirms that surgical excision is the most common treatment modality for all angiosarcomas irrespective of the location in order to achieve complete cure [26,27,66]. Chemotherapy is generally recommended when the surgical treatment is contraindicated and for metastatic disease as a component of palliative therapy [72,73]. A recent article by Ishida et al. [73] summarizes the following treatment strategies for cutaneous angiosarcoma: (1) Surgery followed by radiotherapy is recommended for when the lesion is localized; (2) Paclitaxel is the first-line treatment for advanced tumors; (3) Second-line treatment options include pazopanib and eribulin mesylate; (4) Immunotherapy such as anti-PD1 and PD-L1 may be active against the tumor.
InO-AS could be concluded to be an aggressive tumor, with the literature revealing that 52.2% (22/43) of patients with primary tumors die within 14 months of diagnosis (range of 1–108 months) [10,14,17,18,19,20,21,22]. Except for two patients, all other patients with metastatic tumors died within a period of 14 months (1–36 months) [24,46,47,48,49,50]. Out of the 21 patients who survived, an average survival of 108 months within a range of 1–192 months was recorded [10,11,12,13,15,18,26,30,31,33,34,41,42]. The majority of patients with long-term survival presented with InO-AS on sites such as buccal mucosa and lip [26].

3.2. Kaposi Sarcoma

During the literature review, 20 oral Kaposi sarcoma cases were analyzed. Out of the 20 reported cases, 19 were male patients. All subtypes can present in the oral cavity [7]. Despite the subtypes, a male predilection was observed in Kaposi sarcoma with different male: female ratios. The average age of patients was 44.5 years, ranging from 25 to 80. There were no pediatric patients among the analyzed case reports. In some literature, HIV-related subtype of Kaposi sarcoma was documented as common among pediatric populations [7]. The HIV-related form was the most common (n = 13) subtype reported for oral Kaposi sarcoma. It will be the first presentation of HIV in some cases [51,52]. Presentation is common among male homosexuals who are seronegative for HIV in the Western hemisphere. When immunity deteriorates in HIV patients, Kaposi sarcoma lesions progressively enlarge in size, will multiply, become more nodular or coalesce together [7].
The palate is the most common site of presentation (65%), followed by the maxillary gingiva and mandibular gingiva. Even though oral Kaposi sarcoma with underlying bone erosion is reported in the literature, it is rare [53]. Approximately half of patients with HIV-related KS will show visceral involvement according to the literature. This will be demonstrated with symptoms like vomiting, abdominal pain, and bleeding when the gastrointestinal tract is involved. Pulmonary lesions are considered as potentially life threatening [7]. Primary intraosseous KS and involvement of major salivary glands has been reported in the literature [74,75]. Clinical presentations of the 20 cases analyzed had a nodular variant as the common variant. Treatments for the lesions were antiretroviral therapy (40%) combined with other modalities owing to a high number of HIV-related cases.
It is demonstrated that HHV-8 is associated with all types of KS lesions. There is a complex interaction between the immune system, genetics and environment in causation of the lesion [5]. Histology is similar in different types of KS lesions, and a subtle increase in vascularity in the papillary dermis can be seen on initial lesions. When the lesions progress into the patch type, irregular vascular channels which are parallel to the epidermis dissecting dermal collagen are seen. These are prominent in perivascular and periadnexal tissue, and sometimes spindle cell proliferation is seen in the vicinity of pre-existing vessels. Atypical flat-to-oval-shaped endothelial cells are identified. Hemosiderin and extravasated blood are constantly seen. The above features will exaggerate and become numerous with progression into the plaque stage, with the appearance of hyaline globules (degenerating red blood cells). In the nodular stage, there will be intersecting fascicles of bland spindle cells with elongated nuclei. Slit-like vascular spaces are seen within nodular proliferations (Figure 2). Hyaline globules are periodic acid Schiff (PAS)-positive and diastase-resistant. Latency-associated nuclear antigen (LANA-1) is a highly sensitive and specific marker for KS, with cytoplasmic positivity to CD34, CD31 and D2-40 and nuclear positivity for FLI-1, just like endothelial cells [5].
Treatment of Kaposi sarcoma includes local and systemic therapeutics with significant heterogeneity. Treatments given for these patients include antiretroviral therapy, dose modification of immunosuppression, topical application of imiquimod, interferon therapy, cryotherapy, chemotherapy, surgery, electrochemotherapy and radiotherapy. Currently, there is no unified strategy for treatment of Kaposi sarcoma. Referrals to an appropriate specialist with multidisciplinary approach are important in management and follow-up. Comorbidities are very common during treatments. The iatrogenic type of KS shows higher rate of mortality [76]. During analysis of the literature, it was observed that a combination of multiple treatment modalities was used in treating these patients (Table 3).
In summary, Enzinger et al. reveals age above 50 years, head and neck location, multifocal disease, larger tumors of >5 cm, positive margins, epithelioid histology and necrosis as poor prognostic factors for angiosarcomas of all sites, which could be interpreted as including intraoral tumors as well.
Overall, the complex interplay between the immune system, genetics, and environmental factors in the pathogenesis of Kaposi sarcoma, coupled with the diverse clinical and histological presentations, emphasizes the necessity for tailored and multifaceted therapeutic approaches in managing this malignancy.

4. Conclusions

Although rare, both InO-AS and InO-KS can manifest intraorally without significant differences in behavior compared to their extra oral counterparts. Both malignancies exhibit complex histopathological features, necessitating immunohistochemistry for accurate diagnosis. The treatment often involves surgery, radiotherapy, and chemotherapy, with survival influenced by various factors, including tumor size and stage at diagnosis. However, a notable limitation identified in this review is the inconsistency in histopathological reporting of AS, particularly regarding histopathological grade, which significantly affects overall outcomes. This inconsistency poses a challenge in accurately assessing and comparing treatment outcomes across studies.

Author Contributions

Conceptualization, P.R.J. and R.D.J.; methodology, W.A.M.U.L.A. and P.M.P.; formal analysis, H.A.W.W.; investigation, P.R.J. and H.A.W.W.; data curation, H.A.W.W.; writing—original draft preparation, P.M.P. and W.A.M.U.L.A.; writing—review and editing, P.R.J., L.A.H.J., P.M.P. and W.A.M.U.L.A.; supervision, P.R.J. and R.D.J.; project administration, R.D.J.; All authors have read and agreed to the published version of the manuscript.

Funding

This research received no external funding.

Conflicts of Interest

The authors declare no conflict of interest.

References

  1. Goldblum, J.; Flope, A.; Weiss, S. (Eds.) Malignant Vascular Tumors. In Enzinger and Weiss’s Soft Tissue Tumors; Elsevier: Amsterdam, The Netherlands, 2020; pp. 785–816. [Google Scholar]
  2. Lahat, G.; Dhuka, A.R.; Hallevi, H.; Xiao, L.; Zou, C.; Smith, K.D.; Phung, T.L.; Pollock, R.E.; Benjamin, R.; Hunt, K.K.; et al. Angiosarcoma. Ann. Surg. 2010, 251, 1098–1106. [Google Scholar] [CrossRef]
  3. Stănescu, L.; Foarfă, C.; Georgescu, A.C.; Georgescu, I. Kaposi’s Sarcoma Associated with AIDS. Rom. J. Morphol. Embryol. 2007, 48, 181–187. [Google Scholar]
  4. Jindal, J.R.; Campbell, B.H.; Ward, T.O.; Almagro, U.S. Kaposi’s Sarcoma of the Oral Cavity in a Non-aids Patient: Case Report and Review of the Literature. Head Neck 1995, 17, 64–68. [Google Scholar] [CrossRef] [PubMed]
  5. Flood, L.M. Vascular Lesions of the Head and Neck: Diagnosis and Managementm; Persky, S., Waner, M., Blei, F., Berenstein, A., Eds.; Thieme Publishers: New York, NY, USA, 2014; p. 168. ISBN 978 1 60406 059 1. EISBN 978 1 58890 282 3. [Google Scholar]
  6. Bishop, B.N.; Lynch, D.T. Kaposi Sarcoma; National Library of Medicine: Bethesda, MD, USA, 2023. Available online: https://www.ncbi.nlm.nih.gov/books/NBK534839/ (accessed on 8 June 2024).
  7. Fatahzadeh, M. Kaposi Sarcoma: Review and Medical Management Update. Oral Surg. Oral Med. Oral Pathol. Oral Radiol. 2012, 113, 2–16. [Google Scholar] [CrossRef]
  8. Tounouga, D.N.; Kouotou, E.A.; Nansseu, J.R.; Zoung-Kanyi Bissek, A.-C. Epidemiological and Clinical Patterns of Kaposi Sarcoma: A 16-Year Retrospective Cross-Sectional Study from Yaoundé, Cameroon. Dermatology 2018, 234, 198–204. [Google Scholar] [CrossRef] [PubMed]
  9. Lali, B.S.; Chowdhury, Z.; Gupta, M.; Mishra, A. Primary Angiosarcoma of the Oral Cavity in a Young Adult. Autops. Case Rep. 2021, 11, e2020217. [Google Scholar] [CrossRef] [PubMed]
  10. Nagata, M.; Yoshitake, Y.; Nakayama, H.; Yoshida, R.; Kawahara, K.; Nakagawa, Y.; Shinohara, M. Angiosarcoma of the Oral Cavity: A Clinicopathological Study and a Review of the Literature. Int. J. Oral Maxillofac. Surg. 2014, 43, 917–923. [Google Scholar] [CrossRef] [PubMed]
  11. Terada, T. Angiosarcoma of the Oral Cavity. Head Neck Pathol. 2011, 5, 67–70. [Google Scholar] [CrossRef]
  12. Kusaka, I.; Katagiri, K.; Saito, D.; Ohashi, Y.; Oikawa, S.; Tsuchida, K.; Miyaguchi, J.; Kusaka, T.; Ikeda, R.; Shiga, K. A Case Report of Angiosarcoma Originating from the Tongue. Clin. Case Rep. 2023, 11, e8330. [Google Scholar] [CrossRef]
  13. Komatsu, Y.; Miyamoto, I.; Ohashi, Y.; Katagiri, K.; Saito, D.; Obara, M.; Takeda, Y.; Shiga, K.; Yamada, H. Primary Epithelioid Angiosarcoma Originating from the Mandibular Gingiva: A Case Report of an Extremely Rare Oral Lesion. World J. Surg. Oncol. 2020, 18, 260. [Google Scholar] [CrossRef]
  14. Favia, G.; Lo Muzio, L.; Serpico, R.; Maiorano, E. Angiosarcoma of the Head and Neck with Intra-Oral Presentation. A Clinico-Pathological Study of Four Cases. Oral Oncol. 2002, 38, 757–762. [Google Scholar] [CrossRef] [PubMed]
  15. Terada, T. Angiosarcoma of the Mandibular Gingiva. Int. J. Clin. Exp. Pathol. 2011, 4, 791–793. [Google Scholar]
  16. Fomete, B.; Samaila, M.; Edaigbini, S.; Agbara, R.; Okeke, U.A. Primary Oral Soft Tissue Angiosarcoma of the Cheek: A Case Report and Literature Review. J. Korean Assoc. Oral Maxillofac. Surg. 2015, 41, 273. [Google Scholar] [CrossRef]
  17. Patel, P.B.; Kuan, E.C.; Peng, K.A.; Yoo, F.; Nelson, S.D.; Abemayor, E. Angiosarcoma of the Tongue: A Case Series and Literature Review. Am. J. Otolaryngol. 2017, 38, 475–478. [Google Scholar] [CrossRef] [PubMed]
  18. Harter, J.H. Angiosarcoma of Tongue; Case Report. Laryngoscope 1927, 37, 869–871. [Google Scholar] [CrossRef]
  19. Koteshwer Rao, K.; Nanda Kumar, H.; Rao, R.V. Primary Haemangiosarcoma of the Tongue. Int. J. Oral Maxillofac. Surg. 1986, 15, 489–491. [Google Scholar] [CrossRef]
  20. Frick, W.G.; Keith McDaniel, R. Angiosarcoma of the Tongue: Report of a Case. J. Oral Maxillofac. Surg. 1988, 46, 496–498. [Google Scholar] [CrossRef]
  21. Tabata, M.; Sugihara, K.; Matsui, R.; Yonezawa, S.; Abeyama, K.; Maruyama, I. Angiosarcoma of the Tongue: Report of a Case with Immunohistochemical Findings. J. Oral Pathol. Med. 1999, 28, 92–95. [Google Scholar] [CrossRef]
  22. Yasumatsu, R.; Hirakawa, N.; Tomita, K. Postradiation Angiosarcoma of the Tongue. Eur. Arch. Oto Rhino Laryngol. 2000, 257, 464–465. [Google Scholar] [CrossRef]
  23. Loudon, J.A.; Billy, M.L.; DeYoung, B.R.; Allen, C.M. Angiosarcoma of the Mandible. Oral Surgery Oral Med. Oral Pathol. Oral Radiol. Endodontol. 2000, 89, 471–476. [Google Scholar] [CrossRef]
  24. Fanburg-Smith, J.C.; Furlong, M.A.; Childers, E.L.B. Oral and Salivary Gland Angiosarcoma: A Clinicopathologic Study of 29 Cases. Mod. Pathol. 2003, 16, 263–271. [Google Scholar] [CrossRef] [PubMed]
  25. Oliver, A.J.; Radden, B.G.; Gibbons, S.D.; Busmanis, I.; Cook, R.M. Primary Angiosarcoma of the Oral Cavity. Br. J. Oral Maxillofac. Surg. 1991, 29, 38–41. [Google Scholar] [CrossRef]
  26. Di Battista, M.; Darling, M.R.; Scrivener, E.; Stapleford, R.; Wehrli, B.; McCord, C. Histologic and Immunopathologic Variability in Primary Intraoral Angiosarcoma: A Case Report and Review of the Literature. Head Neck Pathol. 2020, 14, 1139–1148. [Google Scholar] [CrossRef] [PubMed]
  27. Pandey, M.; Thomas, G.; Mathew, A.; Abraham, E.; Somanathan, T.; Ramadas, K.; Iype, E.; Ahamed, I.; Sebastian, P.; Nair, M. Sarcoma of the Oral and Maxillofacial Soft Tissue in Adults. Eur. J. Surg. Oncol. 2000, 26, 145–148. [Google Scholar] [CrossRef] [PubMed]
  28. Olson, M.T.; Puttgen, K.B.; Westra, W.H. Angiosarcoma Arising from the Tongue of an 11-Year-Old Girl with Xeroderma Pigmentosum. Head Neck Pathol. 2012, 6, 255–257. [Google Scholar] [CrossRef] [PubMed]
  29. Sachdeva, S.; Dutta, S.; Rout, P. Angiosarcoma of Mandible: An Uncommon Clinical Entity. Int. J. Appl. Basic Med. Res. 2015, 5, 142–144. [Google Scholar] [CrossRef]
  30. Hunasgi, S. Angiosarcoma of Anterior Mandibular Gingiva Showing Recurrence—A Case Report with Immunohistochemistry. J. Clin. Diagn. Res. 2016, 10, ZD01. [Google Scholar] [CrossRef]
  31. Henny, F.A. Angiosarcoma of the Maxilla in a 3-Month-Old Infant; Report of Case. J. Oral Surg. 1949, 7, 250–252. [Google Scholar]
  32. Blake, H.; Blake, F.S. Angiosarcoma. Oral Surg. Oral Med. Oral Pathol. 1956, 9, 821–825. [Google Scholar] [CrossRef]
  33. Quinn, J.H.; McConnell, H.A.; Leonard, G.L. Multifocal Angiosarcoma of the Gingiva: Report of Case. J. Oral Surg. 1970, 28, 215–217. [Google Scholar]
  34. Albright, C.R.; Shelton, D.W.; Vatral, J.J.; Hobin, F.C. Angiosarcoma of the Gingiva: Report of Case. J. Oral Surg. 1970, 28, 913–917. [Google Scholar] [PubMed]
  35. Piscioli, F.; Leonardi, E.; Scappini, P.; Cristofolini, M.; King, D.F. Primary Angiosarcoma of the Gingiva. Am. J. Dermatopathol. 1986, 8, 430–435. [Google Scholar] [CrossRef] [PubMed]
  36. Kashima, K.; Igakura, Y.; Komura, M.; Hamada, M.; Arima, R.; Sakoda, S. Three Gingival Tumors Derived from Vascular Endothelial Cells: A Case of Hemangioendothelioma and Two Cases of Angiosarcoma. Nihon Koku Shuyo Gakkaishi 1994, 6, 251–261. [Google Scholar]
  37. Margiotta, V.; Florena, A.M.; Giuliana, G. Primary Angiosarcoma of the Alveolar Mucosa in a Haemodialysis Patient: Case Report and Discussion. J. Oral Pathol. Med. 1994, 23, 429–431. [Google Scholar] [CrossRef]
  38. Muñoz, M.; Monje, F.; del Hoyo, J.A.; Martín-Granizo, R. Oral Angiosarcoma Misdiagnosed as a Pyogenic Granuloma. J. Oral Maxillofac. Surg. 1998, 56, 488–491. [Google Scholar] [CrossRef]
  39. Abdullah, B.H.; Yahya, H.I.; Talabani, N.A.; Alash, N.I.; Mirza, K.B. Gingival and Cutaneous Angiosarcoma. J. Oral Pathol. Med. 2000, 29, 410–412. [Google Scholar] [CrossRef] [PubMed]
  40. Florescu, M.; Simionescu, C.; Mărgăritescu, C.; Georgescu, C.V. Gingival Angiosarcoma: Histopathologic and Immunohistochemical Study. Rom. J. Morphol. Embryol. 2005, 46, 57–61. [Google Scholar]
  41. Uchiyama, Y.; Murakami, S.; Kishino, M.; Furukawa, S. A Case Report of Primary Gingival Angiosarcoma. Oral Surgery, Oral Med. Oral Pathol. Oral Radiol. Endodontol. 2009, 108, e17–e21. [Google Scholar] [CrossRef]
  42. Sumida, T.; Murase, R.; Fujita, Y.; Ishikawa, A.; Hamakawa, H. Epulis-like Gingival Angiosarcoma of the Mandible: A Case Report. Int. J. Clin. Exp. Pathol. 2012, 5, 830–833. [Google Scholar]
  43. Aditya, A.; Lele, S. A Nodular Growth on Maxillary Gingiva. Indian J. Dent. Res. 2012, 23, 116. [Google Scholar] [CrossRef]
  44. Tojo, M.; Yoshida, K.; Arakane, N.; Tamagawa, H.; Miyanaga, R.; Watanabe, K.; Fukuhara, S. Primary Angiosarcoma of the Ascending Colon Diagnosed after the Discovery of Intraoral Tumor. Clin. J. Gastroenterol. 2023, 16, 193–197. [Google Scholar] [CrossRef] [PubMed]
  45. Yang, J.; Mei, K.; Wu, C.; Wu, Y.; Xu, Y. Angiosarcoma of the Gingiva: Metastasis from a Primary Tumor of the Scalp. Indian J. Dermatol. Venereol. Leprol. 2017, 83, 626. [Google Scholar] [CrossRef]
  46. Mota, M.E.; Tomo, S.; Alves, F.D.A.; Pellissari, G.A.; do Nascimento, A.G.; Lopes, R.N. Gingival Metastasis of Angiosarcoma of the Breast as a First Manifestation of Spreading Disease: Case Report and Review of the Literature. Spec. Care Dent. 2024, 44, 458–464. [Google Scholar] [CrossRef] [PubMed]
  47. Al Ali, M.M.; Al Otaibi, L.M.; Al Mohaya, M.A.; Khoja, H.A. Intraoral Angiosarcoma with Unusual Clinical Presentation: A Case Report. Heliyon 2023, 9, e17056. [Google Scholar] [CrossRef] [PubMed]
  48. Carr, R.J.; Green, D.M. Oral Presentation of Disseminated Angiosarcoma. Br. J. Oral Maxillofac. Surg. 1986, 24, 277–285. [Google Scholar] [CrossRef]
  49. Tanaka, Y.; Seike, S.; Tomita, K.; Ikeda, J.; Morii, E.; Isomura, E.T.; Kubo, T. Possible Malignant Transformation of Arteriovenous Malformation to Angiosarcoma: Case Report and Literature Review. J. Surg. Case Rep. 2019, 2019. [Google Scholar] [CrossRef]
  50. Hoshimoto, Y.; Aoki, T.; Ichikawa, M.; Ishii, Y.; Kondo, Y.; Uchibori, M.; Sasaki, M.; Naito, H.; Ota, Y. Metastatic Maxillary Gingival Angiosarcoma with Aggressive Growth: A Case Report. Tokai J. Exp. Clin. Med. 2024, 49, 22–26. [Google Scholar]
  51. Sanchez, I.M.; DiTommaso, L.E.; Tsoukas, M.M. Oral Kaposi Sarcoma. JAMA Dermatology 2019, 155, 370. [Google Scholar] [CrossRef]
  52. Sakina, G.; Liew, Y.T. Kaposi’s Sarcoma of the Palate. QJM An Int. J. Med. 2022, 114, 896–897. [Google Scholar] [CrossRef]
  53. Konstantinopoulos, P.A.; Goldsztein, H.; Dezube, B.J.; Pantanowitz, L. Acquired Immunodeficiency Syndrome Related Kaposi’s Sarcoma Eroding the Maxillary Bone. J. Laryngol. Otol. 2008, 122, 993–997. [Google Scholar] [CrossRef]
  54. Martins-de-Barros, A.V.; Carvalho, M.D.V.; Araújo, F.A.D.C. AIDS-Related Kaposi Sarcoma of the Oral Cavity. Rev. Soc. Bras. Med. Trop. 2023, 56, e0133. [Google Scholar] [CrossRef]
  55. Kennedy-LeJeune, E.; Cataldo, V.D. Kaposi’s Sarcoma of the Oral Cavity. N. Engl. J. Med. 2017, 376, 1268. [Google Scholar] [CrossRef]
  56. Wild, R.; Balmer, M.C. Have We Forgotten? Oral Manifestations of Kaposi’s Sarcoma. Sex. Transm. Infect. 2015, 91, 345. [Google Scholar] [CrossRef]
  57. Pugalagiri, P.; Muller, S.; Cox, D.P.; Kessler, H.P.; Wright, J.M.; Cheng, Y.-S.L. Lymphangioma-like Kaposi Sarcoma of the Oral Mucosa. Oral Surg. Oral Med. Oral Pathol. Oral Radiol. 2013, 116, 84–90. [Google Scholar] [CrossRef] [PubMed]
  58. Lombardi, N.; Varoni, E.; Sardella, A.; Lodi, G. Oral Kaposi’s Sarcoma in a HIV-Negative Young Patient. Oral Oncol. 2020, 103, 104567. [Google Scholar] [CrossRef]
  59. Wu, Y.-H.; Yang, H.; Sun, A.; Chen, H.-M. Kaposi’s Sarcoma of the Hard Palate. J. Formos. Med. Assoc. 2016, 115, 883–884. [Google Scholar] [CrossRef]
  60. Faden, A.; AlSheddi, M.; AlKindi, M.; Alabdulaaly, L. Oral Kaposi Sarcoma in HIV-Seronegative Saudi Patient: Literature Review and Case Report. Saudi Dent. J. 2017, 29, 129–134. [Google Scholar] [CrossRef]
  61. Safia, A.; Farhat, R.; Avraham, Y.; Merchavy, S. Kaposi Sarcoma at the Base of the Tongue in a Renal Transplant Patient. BMJ Case Rep. 2023, 16, e253899. [Google Scholar] [CrossRef] [PubMed]
  62. Howard, J.H.; Darrow, M.; Chen, L.; Alnimri, M.; Jen, K. Tonsillar Kaposi Sarcoma in a Renal Transplant Patient. Transpl. Infect. Dis. 2020, 22, e13347. [Google Scholar] [CrossRef] [PubMed]
  63. Lorusso, F.; Di Vincenzo, S.A.; Campofiorito, V.; Sireci, F.; Immordino, A.; Dispenza, F. Tonsillar Kaposi’s Sarcoma in HIV Positive Patient with Syphilis Infection. Iran. J. Otorhinolaryngol. 2024, 36, 437–441. [Google Scholar]
  64. Feller, L.; Anagnostopoulos, C.; Wood, N.H.; Bouckaert, M.; Raubenheimer, E.J.; Lemmer, J. Human Immunodeficiency Virus–Associated Kaposi Sarcoma as an Immune Reconstitution Inflammatory Syndrome: A Literature Review and Case Report. J. Periodontol. 2008, 79, 362–368. [Google Scholar] [CrossRef]
  65. Papagatsia, Z.; Jones, J.; Morgan, P.; Tappuni, A.R. Oral Kaposi Sarcoma: A Case of Immune Reconstitution Inflammatory Syndrome. Oral Surgery Oral Med. Oral Pathol. Oral Radiol. Endodontol. 2009, 108, 70–75. [Google Scholar] [CrossRef] [PubMed]
  66. Lee, K.C.; Chuang, S.-K.; Philipone, E.M.; Peters, S.M. Characteristics and Prognosis of Primary Head and Neck Angiosarcomas: A Surveillance, Epidemiology, and End Results Program (SEER) Analysis of 1250 Cases. Head Neck Pathol. 2019, 13, 378–385. [Google Scholar] [CrossRef] [PubMed]
  67. Nakano, Y.; Sazumi, Y.; Mizuta, Y.; Sakae, H.; Otsuka, F. Gingival Lesion Leading to a Diagnosis of Angiosarcoma. J. Gen. Fam. Med. 2021, 22, 90–91. [Google Scholar] [CrossRef] [PubMed]
  68. Eslami, A.; Miyaguchi, K.; Mogushi, K.; Watanabe, H.; Okada, N.; Shibuya, H.; Mizushima, H.; Miura, M.; Tanaka, H. PARVB Overexpression Increases Cell Migration Capability and Defines High Risk for Endophytic Growth and Metastasis in Tongue Squamous Cell Carcinoma. Br. J. Cancer 2015, 112, 338–344. [Google Scholar] [CrossRef] [PubMed]
  69. Mullins, B.; Hackman, T. Angiosarcoma of the Head and Neck. Int. Arch. Otorhinolaryngol. 2015, 19, 191–195. [Google Scholar] [CrossRef]
  70. Mark, R.J.; Tran, L.M.; Sercarz, J.; Fu, Y.S.; Calcaterra, T.C.; Juillard, G.F. Angiosarcoma of the Head and Neck: The UCLA Experience 1955 Through 1990. Arch. Otolaryngol. Head Neck Surg. 1993, 119, 973–978. [Google Scholar] [CrossRef]
  71. Lee, N.C.J.; Eskander, A.; Miccio, J.A.; Park, H.S.; Shah, C.; Rutenberg, M.; Hosni, A.; Husain, Z.A. Evaluation of Head and Neck Soft Tissue Sarcoma 8th Edition Pathologic Staging System and Proposal of a Novel Stage Grouping System. Oral Oncol. 2021, 114, 105137. [Google Scholar] [CrossRef]
  72. Houpe, J.E. Treatment of Angiosarcoma of the Head and Neck: A Systematic Review. Cutis 2023, 111, 247–251. [Google Scholar] [CrossRef]
  73. Ishida, Y.; Otsuka, A.; Kabashima, K. Cutaneous Angiosarcoma: Update on Biology and Latest Treatment. Curr. Opin. Oncol. 2018, 30, 107–112. [Google Scholar] [CrossRef]
  74. Nichols, C.M.; Flaitz, C.M.; Hicks, M.J. Primary Intraosseous Kaposi’s Sarcoma of the Maxilla in Human Immunodeficiency Virus Infection. J. Oral Maxillofac. Surg. 1995, 53, 325–329. [Google Scholar] [CrossRef] [PubMed]
  75. Castle, J.T.; Thompson, L.D.R. Kaposi Sarcoma of Major Salivary Gland Origin. Cancer 2000, 88, 15–23. [Google Scholar] [CrossRef]
  76. Russo, I.; Marino, D.; Cozzolino, C.; Del Fiore, P.; Nerjaku, F.; Finotto, S.; Cattelan, A.; Calabrò, M.L.; Belloni Fortina, A.; Russano, F.; et al. Kaposi’s Sarcoma: Evaluation of Clinical Features, Treatment Outcomes, and Prognosis in a Single-Center Retrospective Case Series. Cancers 2024, 16, 691. [Google Scholar] [CrossRef] [PubMed]
Jvd 03 00024 g001
Figure 1. (A,B): Photomicrographs showing spindle cell differentiation. (A): 10×, (B): 20×, (C): 40× high-power view to showing pleomorphic tumor cells. (D): 10× tumor composed of epithelioid cells showing high degree of cytological atypia. Necrosis is present towards the right side. (Obtained from the archives of Department of Oral Pathology, Faculty of Dental Sciences, University of Peradeniya, Sri Lanka.)
Figure 1. (A,B): Photomicrographs showing spindle cell differentiation. (A): 10×, (B): 20×, (C): 40× high-power view to showing pleomorphic tumor cells. (D): 10× tumor composed of epithelioid cells showing high degree of cytological atypia. Necrosis is present towards the right side. (Obtained from the archives of Department of Oral Pathology, Faculty of Dental Sciences, University of Peradeniya, Sri Lanka.)
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Figure 2. (A–D): Histopathological presentations of InO-KS occurring in a male. Note: Nodular presentation, spindle cells arranged into short fascicles and slit-like vascular spaces (Courtesy of Dr. S. Wadusinghearachchi, National Dental Hospital, Colombo, Sri Lanka).
Figure 2. (A–D): Histopathological presentations of InO-KS occurring in a male. Note: Nodular presentation, spindle cells arranged into short fascicles and slit-like vascular spaces (Courtesy of Dr. S. Wadusinghearachchi, National Dental Hospital, Colombo, Sri Lanka).
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Table 1. Details of the three unpublished cases diagnosed as angiosarcomas.
Table 1. Details of the three unpublished cases diagnosed as angiosarcomas.
Clinical PresentationHistopathologyImmunohistochemistry
Case 149-year-old male
Three-month history of a growth on the right side of the cheek
Intraoral examination: reddish-pink exophytic growth (6.0 × 4.0 cm2) on the right buccal mucosa
Firm and non-tender lesion
No palpable cervical lymph nodes		Tumor composed of anastomosing vascular spaces. Lined by atypical plump endothelial cells, predominantly epithelioid. Numerous mitoses with abnormal forms. Minimal collagenous stroma. CD31& CD34: Strongly positive in most tumor cells. EMA & Pan CK: focally positive. S100, Desmin, SMA, Caldesmon:
Negative for tumor cells
Case 259-year-old male
Ulcerative growth over the left side floor of the mouth
Impaired tongue movements
Intraoral examination: 1.0 × 0.6 cm2 wedge-shaped red-colored growth with soft consistency
Painful ulceration with everted edges		Unencapsulated tumor with high degree of cytological atypia. Marked cellular and nuclear pleomorphism in some tumor islands. Intracytoplasmic lumina formation in some tumor cells.Factor VIII: Positive in tumor cells
Cytokeratin: Positive in tumor cells
Case 361-year-old male
Rapidly growing painless gingival swelling on the right side of the lower alveolar region two weeks after tooth extraction		Unencapsulated tumor composed of discohesive sheets and islands of epithelial cells. Large vesicular nuclei, eosinophilic cytoplasm, high-grade nuclear pleomorphism, increased mitotic activity. Decalcified specimen of bone margins showed evidence of the tumor. Four out of eight lymph nodes from submandibular gland specimen showed extensive tumor metastasis and necrosis with extracapsular invasion.CK (Cytokeratin) and CD31 positive in tumor cells, MyoD1 and CK 5/6 negative
Table 2. Clinicopathological presentations of primary and metastatic angiosarcoma of oral cavity.
Table 2. Clinicopathological presentations of primary and metastatic angiosarcoma of oral cavity.
FeaturePrimary Angiosarcoma
(n = 60)		Metastatic Angiosarcoma
(n = 14)
Age in years
0–18
19–64
65
11.67% (n = 7)
51.67% (n = 31)
36.66% (n = 22)
-
42.86% (n = 6)
57.14% (n = 8)
Sex
Male
Female
56.67% (n = 34)
43.33% (n = 26)
78.57% (n = 11)
21. 43% (n = 3)
Site
Tongue
Buccal mucosa
Lip
Palate
Mandibular gingiva/alveolar mucosa
Maxillary gingiva/alveolar mucosa
Both (mandibular gingiva + maxillary gingiva)
35.00% (n = 21)
6.67% (n = 4)
6.67% (n = 4)
6.67% (n = 4)
31.67% (n = 19)
10.00% (n = 6)
1.67% (n = 1)




28. 57% (n = 4)
64.29% (n = 9)
7.14% (n = 1)
Bone involvement
Yes
No
38.33% (n = 23)
61.67% (n = 37)
71.43% (n = 10)
21.43% (n = 3)
Size
>5 cm
<5 cm
Not available
23.33% (n = 14)
26.67% (n = 16)
50.00% (n = 30)
-
42.86% (n = 6)
57.14% (n = 8)
Treatment
Surgery only (S)
Radiotherapy only (R)
Chemotherapy only (C)
S + R + C
S + R
S + C
R + C
33.33% (n = 20)
1.67% (n = 1)
1.67% (n = 1)
1.67% (n = 1)
11.67% (n = 7)
6.67% (n = 4)
5.00% (n = 3)
7.14% (n = 1)
7.14% (n = 1)
7.14% (n = 1)

7.14% (n = 1)
7.14% (n = 1)
14.28% (n = 2)
Outcome
NED
DOD
Not available
DOC
35.00% (n = 21)
36.606% (n = 22)
26.66% (n = 16)
1.67% (n = 1)
14.28% (n = 2)
71.43% (n = 10)
14.28% (n = 2)
NED = No evidence of disease, DOD = Dead of disease, DOC = Died due to other cause.
Table 3. Clinicopathological presentations of Kaposi’s sarcoma of oral cavity [3,50,51,52,53,54,55,56,57,58,59,60,61,62,63,64].
Table 3. Clinicopathological presentations of Kaposi’s sarcoma of oral cavity [3,50,51,52,53,54,55,56,57,58,59,60,61,62,63,64].
FeatureKaposi’s Sarcoma (n = 20)
Age in years
0–18-
19–6485.00% (n = 17)
6510.00% (n = 2)
N/A5.00% (n = 1)
Sex
Male95.00% (n = 19)
Female5.00% (n = 1)
Site
Tongue5.00% (n = 1)
Palate65.00% (n = 13)
Mandibular gingiva15.00% (n = 3)
Maxillary gingiva40.00% (n = 8)
Tonsils10. 00% (n = 2)
Retromolar area10.00% (n = 2)
Mandible5.00% (n = 1)
Bone involvement
Yes 25.00% (n = 5)
No 30.00% (n = 6)
Not mentioned45.00% (n = 9)
Size
>5 cm5.00% (n = 1)
<5 cm20.00% (n = 4)
Not available75.00% (n = 15)
Treatment
Surgery (S)5.00% (n = 1)
Radiotherapy15. 00% (n = 3)
Anti-retroviral therapy (ART)10.00% (n = 2)
ART + S10.00% (n = 2)
ART + Chemotherapy (C)10.00% (n = 2)
ART + S + C10.00 (n = 2)
R + C (palliative) 5.00 (n = 1)
Outcome
NED *45.00% (n = 9)
DOD **-
Partial regression10.00% (n = 2)
Not available45.00 (n = 9)
Type
HIV65.00% (n = 13)
Iatrogenic
Immunocompromised (DM) 5.00% (n = 1)
Immunosuppressed10.00% (n = 2)
Classic15.00% (n = 3)
Not indicated5.00% (n = 1)
Clinical presentation
Nodule65.00% (n = 13)
Plaque10.00% (n = 2)
Ulcerated mass10.00% (n = 2)
Soft tissue swelling 15.00% (n = 3)
* NED = No evidence of Disease. ** DOD = Dead of Disease.
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Jayasooriya, P.R.; Weerasinghe, H.A.W.; Jayasinghe, L.A.H.; Peiris, P.M.; Abeyasinghe, W.A.M.U.L.; Jayasinghe, R.D. A Comprehensive Literature Review on Diagnostic Strategies and Clinical Outcome of Intraoral Angiosarcoma and Kaposi Sarcoma. J. Vasc. Dis. 2024, 3, 306-318. https://doi.org/10.3390/jvd3030024

AMA Style

Jayasooriya PR, Weerasinghe HAW, Jayasinghe LAH, Peiris PM, Abeyasinghe WAMUL, Jayasinghe RD. A Comprehensive Literature Review on Diagnostic Strategies and Clinical Outcome of Intraoral Angiosarcoma and Kaposi Sarcoma. Journal of Vascular Diseases. 2024; 3(3):306-318. https://doi.org/10.3390/jvd3030024

Chicago/Turabian Style

Jayasooriya, Primali Rukmal, Hiruni Ashcharya Wijerathna Weerasinghe, Liyanaarachchige Anushan Hiranya Jayasinghe, Prasangi Madubhashini Peiris, Wijeyapala Abeyasinghe Mudiyanselage Udari Lakshika Abeyasinghe, and Ruwan Duminda Jayasinghe. 2024. "A Comprehensive Literature Review on Diagnostic Strategies and Clinical Outcome of Intraoral Angiosarcoma and Kaposi Sarcoma" Journal of Vascular Diseases 3, no. 3: 306-318. https://doi.org/10.3390/jvd3030024

APA Style

Jayasooriya, P. R., Weerasinghe, H. A. W., Jayasinghe, L. A. H., Peiris, P. M., Abeyasinghe, W. A. M. U. L., & Jayasinghe, R. D. (2024). A Comprehensive Literature Review on Diagnostic Strategies and Clinical Outcome of Intraoral Angiosarcoma and Kaposi Sarcoma. Journal of Vascular Diseases, 3(3), 306-318. https://doi.org/10.3390/jvd3030024

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