Primary malignant melanoma of the urethra: a systematic analysis of the current literature.

BACKGROUND: Primary malignant melanoma of the urethra is a rare tumour that most commonly affects the meatus and distal urethra. General prognosis seems to be poor. To date, there have been no systematic reviews on this topic. Therefore, we aimed to gain more insight into this rare type of tumour. METHODS: Medline and PubMed were searched and all cases of urethral melanoma reported as single case reports or small case series were reviewed as the first step in the combined analyses of all the cases reported. RESULTS: We reviewed 150 cases. The most frequent presentation was urethral mass. The first line of treatment was surgery such as tumour excision or total urethrectomy. Three times more women were diagnosed than men with a mean age of 64.7 years (SD 10.7; median 65.5; range 28-96 years). Forty-three patients (36 %) underwent adjuvant treatment including chemotherapy, radiotherapy or immunotherapy. Recurrences were observed in 71.4 % (n = 80) of the cases, mainly local recurrences (n = 44; 55 %) and metastases in the inguinal lymph nodes (n = 31; 28 %). Recurrences occurred within 12.5 months on average (SD 12.6; range 1-48). The treatments of recurrences were surgery, chemotherapy, radiotherapy and immunotherapy. These treatment options influenced the course of the disease. CONCLUSIONS: Urethral melanoma is mainly a disease of older people with an average age of 64 years. The study showed that the T-stage as a basis of depth invasion is a prognostic factor for urethral melanoma. Moreover, pulmonary metastases (cM2), local recurrence and systemic recurrence influence prognosis. The T-stage classification is useful because of its prognostic ability.

Small cell carcinoma of the ovary of the hypercalcaemic type: an analysis of clinical and prognostic aspects of a rare disease on the basis of cases published in the literature.

PURPOSE: Ovarian small cell carcinoma of the hypercalcaemic type is a very rare and highly aggressive malignant disease, mainly affecting young women. Due to the rarity of this tumour entity, prospective randomised trials are unlikely to be conducted, and the only retrospective analysis based on a large case series is from 1994. Since diagnostic and treatment modalities may have changed, we initiated this analysis. METHODS: The aim of our study was to review and analyse cases published since 1975 to validate former findings and to gather more information about therapy options, diagnostic and prognostic factors. A systematic literature search of the PubMed/Medline database was performed assessing all articles until September 2010. All retrieved articles were evaluated and cross-checked for references on the topic. In total, 135 cases were included, selected from 62 case reports and smaller case studies. RESULTS: Small cell carcinoma mostly affects women with a mean age of 23.4 years. They present with unspecific symptoms like abdominal pain or palpable mass, sometimes accompanied by an elevated calcium or CA-125 serum concentration. The tumour appears nearly almost unilaterally, mostly affecting the right ovary. Tumour stage is a clearly prognostic factor. Adjuvant chemotherapy consisting of etoposide, cisplatinum/carboplatinum or vinca alkaloids has shown improved survival, whereas radiotherapy has not. CONCLUSIONS: In spite of limitations this analysis provides new insights especially with respect to therapeutic aspects. This review underlines the importance of case reports in rare tumour entities in order to answer open questions.