Multidisciplinary Management of Rare Head and Neck Tumors: The Importance of Knowing and Dealing with Them

A special issue of Biomedicines (ISSN 2227-9059). This special issue belongs to the section "Cancer Biology and Oncology".

Deadline for manuscript submissions: 30 September 2025 | Viewed by 4505

Special Issue Editor


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Istituto Nazionale Tumori di Napoli, IRCCS “G. Pascale”, Via Mariano Semmola, 80131 Napoli, Italy
Interests: head and neck oncology; immune-therapy; targeted therapy; pain therapy; electrochemotherapy; cancer diagnostics; treatment tumors; cancer biology; cancer biomarkers; tumor biology cancer; cell biology
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Special Issue Information

Dear Colleagues,

Head and neck tumors represent about 7–8% of all malignancies, and nearly 90% of them have squamous histology. Excluding nonmelanoma skin cancer, the remaining 10% of head and neck carcinomas may arise from the salivary gland, nasopharynx (nonsquamous), and nasal cavity/paranasal sinuses (nonsquamous). Additionally, there are also rare tumors in the head and neck area different from those above, and given their particular rarity they could be defined as rate tumors of the head and neck (RTsHN). These may have different histologies and different embryonic derivations. The management of RTsHN is challenging since, often, no available guidelines are present.

In recent years, advancements in molecular profiling and genomic characterization have provided invaluable insights into the underlying biology of rare head and neck cancers, unravelling novel therapeutic targets and paving the way for precision medicine approaches; however, significant gaps persist in our understanding of these malignancies, hindering progress towards improved outcomes. Through a meticulous review of the existing literature, coupled with insights gleaned from clinical practice and translational research, we endeavor to elucidate the etiopathogenesis, clinical characteristics, diagnostic modalities, treatment strategies, and prognostic factors pertaining to these malignancies.

Dr. Francesco Perri
Guest Editor

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Keywords

  • rare head and neck cancers
  • translational research
  • therapeutic targets

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Published Papers (3 papers)

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Research

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19 pages, 1035 KiB  
Article
Prognostic Factors and Long-Term Outcome Prediction in Patients with Hypopharyngeal Carcinoma Treated with (Chemo)radiotherapy: Development of a Prognostic Model
by Miloslav Pala, Pavla Novakova, Adam Tesar, Lucie Vesela, Antonin Vrana, Jarmila Sukova, Zdenka Pechacova, Petra Holeckova, Tereza Drbohlavova, Tomas Podlesak and Petra Tesarova
Biomedicines 2025, 13(2), 417; https://doi.org/10.3390/biomedicines13020417 - 9 Feb 2025
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Abstract
Background/Objectives: To evaluate the effectiveness of curative (chemo)radiotherapy in patients with hypopharyngeal carcinoma and to identify prognostic factors influencing treatment outcomes. Methods: We conducted a retrospective study of 173 consecutive patients, treated with definitive or postoperative (chemo)radiotherapy from 2002 to 2020 [...] Read more.
Background/Objectives: To evaluate the effectiveness of curative (chemo)radiotherapy in patients with hypopharyngeal carcinoma and to identify prognostic factors influencing treatment outcomes. Methods: We conducted a retrospective study of 173 consecutive patients, treated with definitive or postoperative (chemo)radiotherapy from 2002 to 2020 [median age 60 years; current/former smokers 95%; UICC stage III/IV 96%]. Radiation therapy was preceded by a radical resection of a primary tumor in 32% of patients. One hundred patients received chemotherapy. Results: The median total dose of radiotherapy achieved was 70 Gy. The five- and ten-year locoregional controls were 63%, and the five- and ten-year distant controls were 77% and 76%, respectively. The five- and ten-year overall survival rates were 24% and 9%, respectively. Conclusions: The results demonstrate the limited effectiveness of curative (chemo)radiotherapy in patients with hypopharyngeal carcinoma with long-term locoregional and distant control of half of the treated patients. The multivariate analysis indicated that initial surgery, chemotherapy, comorbidity score (as assessed by ACE-27), pretreatment tracheostomy, hemoglobin level and initial response to treatment were the strongest prognostic factors in predicting survival. Using these factors, corresponding predictive models were constructed. Full article
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14 pages, 5882 KiB  
Article
Long-Term Outcomes of Esophageal Squamous Neoplasia with Muscularis Mucosa Involvement after Endoscopic Submucosal Dissection
by Chen-Huan Yu, Yueh-Hung Chou, Tze-Yu Shieh, Chao-Yu Liu, Jiann-Ming Wu, Chen-Hsi Hsieh, Tzong-Hsi Lee and Chen-Shuan Chung
Biomedicines 2024, 12(8), 1660; https://doi.org/10.3390/biomedicines12081660 - 25 Jul 2024
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Abstract
Ambiguity exists over treatment and surveillance strategies after endoscopic submucosal dissection (ESD) for esophageal squamous cell neoplasia (ESCN) with unfavorable histologic features. This study investigated the long-term outcomes of ESD in high-risk ESCN patients. We retrospectively included early ESCN patients treated with ESD [...] Read more.
Ambiguity exists over treatment and surveillance strategies after endoscopic submucosal dissection (ESD) for esophageal squamous cell neoplasia (ESCN) with unfavorable histologic features. This study investigated the long-term outcomes of ESD in high-risk ESCN patients. We retrospectively included early ESCN patients treated with ESD at two medical centers in Taiwan between August 2010 and December 2023. Demographic, endoscopic and pathological data were collected. Among 146 patients (mean age 59.17 years) with 183 lesions, 73 (50%) had a history of head and neck cancer (HNC). En bloc and R0 resections were achieved in 100% and 95.6% of the lesions, respectively. The 5-year overall survival (OS), disease-specific survival (DSS) and local recurrence rates were 42.7%, 94.7% and 11%. R0 resections were significantly associated with recurrence in a univariate analysis (HR: 0.19, 95% CI: 0.06–0.66, p = 0.008). Alcohol abstinence was independently associated with lower recurrence (HR: 0.34, 95% CI: 0.16–0.73, p = 0.006). Patients with pT1a-MM (muscularis mucosa invasion) had comparable OS (p = 0.82), DSS (p = 0.617) and recurrence (p = 0.63) rates to those with pT1a-EP/LPM (epithelium/lamina propria invasion). The long-term outcomes of ESCN patients after ESD for expanded indications were satisfactory. ESD could be considered in selected ESCN patients involving the muscularis mucosa, notably among high-risk HNC patients. Full article
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Review

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15 pages, 1025 KiB  
Review
Hypothalamic Hamartomas: A Narrative Review
by Marian Mitrica, Aida Mihaela Manole, Mihai Toma, Octavian Mihai Sirbu, Anca Maria Sirbu and Alice Elena Munteanu
Biomedicines 2025, 13(2), 371; https://doi.org/10.3390/biomedicines13020371 - 5 Feb 2025
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Abstract
Hypothalamic hamartomas (HH) are infrequent, non-neoplastic malformations of the hypothalamus with heterogeneous clinical features, with symptoms including gelastic seizures, central precocious puberty, and cognitive or behavioral deficits. This narrative review synthesizes current knowledge regarding the etiology, clinical manifestations, diagnostic advances, and therapeutic approaches [...] Read more.
Hypothalamic hamartomas (HH) are infrequent, non-neoplastic malformations of the hypothalamus with heterogeneous clinical features, with symptoms including gelastic seizures, central precocious puberty, and cognitive or behavioral deficits. This narrative review synthesizes current knowledge regarding the etiology, clinical manifestations, diagnostic advances, and therapeutic approaches for HH. Genetic insights highlight the role of postzygotic mosaicism and dysregulated Sonic Hedgehog signaling in HH development, emphasizing their relevance in potential therapeutic strategies. Diagnostic modalities such as MRI, PET, and SEEG are pivotal in identifying and characterizing HHs, enabling precise treatment planning. Therapeutic interventions span pharmacological, surgical, and neuromodulatory approaches. While surgical approaches, such as transcallosal resection or stereotactic radiosurgery, can offer considerable seizure control, newer modalities, such as laser interstitial laser thermal therapy (LITT) as well as stereotactic radiofrequency thermocoagulation, prioritize minimizing both cognitive and behavioral sequelae. The use of pharmacologic management and neuromodulation provides adjuvant benefits, specifically in drug-resistant epilepsy; despite progress, limitations still remain, including variability of outcomes and not enough long-term studies. This review underscores the need for multidisciplinary care and advanced research to optimize outcomes and improve the quality of life for patients with HH. Full article
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