Search Results (6)

Background/Purpose: Primary urethral cancer is a rare malignancy, accounting for less than 1% of all urogenital cancers. Current epidemiological data from Europe are scarce and outdated. Therefore, the analyzes and comparison of the incidence and mortality of PUC in selected European countries, with particular focus on Poland, based on the most recent available registry data, were performed. Methods: Our study is based on country-level data and is descriptive in nature. Incidence data for PUC were obtained from the national cancer registries of Poland, Latvia, Slovenia, and Hungary. Mortality data were sourced from the WHO Mortality Database. Age-standardized incidence rates were calculated for two time intervals (2000–2009 and 2010–2019). Age-standardized mortality rates for individuals aged ≥45 years were calculated using the European Standard Population (ESP2013). Trends in incidence and mortality in Poland were analyzed using a five-year moving average. Results: The highest incidence of PUC was observed in Hungary, while Poland showed the lowest incidence. Latvia had the highest ASMRs for both sexes, whereas Poland and Greece reported the lowest mortality rates. Despite slight annual fluctuations, the overall PUC mortality rate in Poland has remained stable. Our study is limited by the relatively short analyzed period (2000–2021), restricted availability of C68.0 incidence data from national cancer registries, and incomplete mortality data in the WHO mortality database. Conclusions: This first contemporary comparative analysis of PUC epidemiology in Europe highlights the rarity of this malignancy and the limited data availability. Based on the knowledge drawn from the literature presented in the article on the impact of centralization on the increase in overall survival and the decrease in mortality in rare cancers, the authors believe that centralization of care can improve PUC patient outcomes. Full article

Renal cell carcinoma is the most common type of primary urogenital cancer, usually resistant to radiotherapy and chemotherapy. Hypertrophic lichen planus is an inflammatory dermatosis characterized by the presence of papulosquamous and intensely pruritic lesions. The association of these two conditions is unusual, being reported in the specialized literature only in a few rare cases with the onset of lichenoid lesions after patients have undergone various forms of treatment. The case of a 62-year-old male patient who was admitted for severe abdominal pain due to a giant renal tumor associated with a hypertrophic plaque located on the anterior part of the left calf is presented. After (clinical, biochemical, imaging) diagnosis, surgery was performed for en bloc removal of the entire mass, adrenal gland, and spleen. The histopathological exam established the diagnosis of a moderately differentiated T2b clear cell Grawitz tumor, without regional lymph node metastasis (stage II). The patient continued local corticosteroid therapy in the hospital for hypertrophic lichen planus lesions, being referred to the oncology department after discharge. Full article

Angioleiomyoma, a rare variant of leiomyoma, is a benign tumor of mesenchymal origin. Angioleiomyomas of the female urogenital tract are extremely rare, with only six cases of uterine cervical angioleiomyoma previously reported in the literature. In this case study, we report on a 49-year-old female patient who presented with menorrhagia whose initial magnetic resonance imaging (MRI) findings suggested cervical squamous cell carcinoma (SCC). However, following the hysterectomy, histological examination confirmed the lesion to be angioleiomyoma. To the best of our knowledge, there have been no previously reported cases of angioleiomyomas presenting with MRI findings that are suggestive of uterine SCC. Recognizing that angioleiomyomas can mimic uterine malignancies on MRI may prove beneficial for future diagnostic and treatment strategies. Full article

Uterine leiomyomas usually arise from the uterine body (95%), and rarely from the cervix (0.6%) or other urogenital sites. Lipoleiomyomas are benign, uncommon variants of leiomyomas (0.03–0.2%), histologically composed of smooth muscle cells and mature adipocytes; they usually occur in the uterine body and exceptionally in the cervix. We performed the first systematic literature review of cervical lipoleiomyomas (PRISMA guidelines), presenting five new cases. Including our series, thirty-one detailed cases were reported in the literature (mainly in Asia). The age range was 35–74 years, revealing a higher mean age than conventional cervical leiomyomas (46.5 vs. 39.4 years). Patients were usually multiparous (94%), typically complaining of vaginal bleeding (11/31, 36%), pelvic/abdominal pain (10/31, 32%), and/or urinary disturbances (6/31, 19%) 1 week to 10 months before presentation. Clinical examination revealed a pedunculated tumor (48%), or prolapse of ≥1 pelvic organs (16%). Twenty-four (77%) patients underwent total hysterectomy ± additional surgery; simple myomectomy/excision was performed in five (16%) cases. Only one (3%) of our cases recurred 2 years after partial excision; no evidence of disease was found 13 years after recurrence excision. Adipocytes occupied ≤50% of the tumor volume. Hyaline or myxoid changes and cartilaginous metaplasia were uncommon histological findings. Surgically challenging cases or pregnant patients may require expert gynecologists. Interventional radiology or conservative treatments were rarely proposed. Full article

Mucosal melanomas (MM) are rare tumors, being less than 2% of all diagnosed melanomas, comprising a variegated group of malignancies arising from melanocytes in virtually all mucosal epithelia, even if more frequently found in oral and sino-nasal cavities, ano-rectum and female genitalia (vulva and vagina). To date, there is no consensus about the optimal management strategy of MM. Furthermore, the clinical rationale of molecular tumor characterization regarding BRAF, KIT or NRAS, as well as the therapeutic value of immunotherapy, chemotherapy and targeted therapy, has not yet been deeply explored and clearly established in MM. In this overview, focused on anorectal and genital MM as models of rare melanomas deserving of a multidisciplinary approach, we highlight the need of referring these patients to centers with experts in melanoma, anorectal and uro-genital cancers treatments. Taking into account the rarity, the poor outcomes and the lack of effective treatment options for MM, tailored research needs to be promptly promoted. Full article

Frasier syndrome is a rare disease that affects the kidneys and genitalia. Patients who have Frasier syndrome develop nephrotic syndrome (NS) featuring focal segmental glomerulosclerosis (FSGS) that is resistant to steroid treatment in early childhood. Male patients can have female external genitalia (pseudo-hermaphroditism) at birth and develop gonado-blastoma in their adolescence. Frasier syndrome is caused by mutations in the splice donor site at intron 9 of the Wilms’ tumor WT1 gene; these mutations result in an imbalanced ratio of WT1 protein isoforms and affect the development of the urogenital tract, podocyte function, and tumor suppression. Here, we report on a patient with long-term refractory NS who developed a malignant mixed germ cell tumor arising in a gonado-blastoma of the ovary 8 years after the onset of proteinuria. Full article