Microcystic adnexal carcinoma: report of seven cases including one with lung metastasis.

BACKGROUND: Microcystic adnexal carcinoma (MAC) is a rare cutaneous neoplasm, with a high rate of local recurrences. OBJECTIVE: A series of MAC was analyzed and compared to previously published cases. METHODS: Seven cases of MAC were identified in the register of the institution. Medical and pathological records were reviewed. RESULTS: The primary MAC were located on the face in all patients, and 85% were initially misdiagnosed. The mean follow-up duration was 108 months. The recurrence rate was high: 4 patients developed recurrences. In 3 patients, the course of the disease was severe: one of them developed pathologically proven lung metastasis. CONCLUSION: The present study and review of the literature confirm the clinically aggressive evolution of MAC and its rare ability to give rise to metastasis. Long-term clinical follow-ups with imaging investigations are mandatory.

[Melanoma of the penis: 6 cases]. / Mélanomes du pénis: 6 cas.

BACKGROUND: Melanomas of the penis are rare tumors of poor prognosis. METHODS: Six cases of melanoma of the penis, followed between 1975 and 2002, were retrospectively reviewed. We collected the data on epidemiological, clinical and pathologic factors, treatment and follow-up. RESULTS: The mean age was 44 years. The time to diagnosis was 2 years. Two patients had general predisposing factors for melanoma, and 3 patients local predisposing factors. Two patients had partial penectomy and 4 patients had conservative excision. One patient had local recurrence, and another had metastatic course resulting in death. Five patients out of 6 were alive and disease free at time of the study, with a 24 month follow-up. DISCUSSION: The identified risk factors for the development of penile melanoma are melanosis and pre-existing nevus. Delayed diagnosis explains the usually bad prognosis. Classical surgical treatment used to be radical, but recently, conservative surgery has been proposed. For an early diagnosis, genital melanosis requires surgical excision, when technically feasible, and any atypical lesion of the penis should be submitted to a biopsy.

[Epithelioid sarcoma manifesting as chronic plantar arch ulceration]. / Ulcération chronique de la voûte plantaire révélant un sarcome épithélioïde.

BACKGROUND: Epitheloid sarcoma is an uncommon malignant soft tissue tumor observed in the distal extremities of young men. We report a case of long standing ulceration of the sole which was found to be an epithelioid sarcoma. CASE REPORT: A 78-year-old woman had an indolent ulceration of the left sole for several months. Physical examination disclosed a well demarcated 3-cm ulcerated lesion with a red center and flat edges. Skin sections confirmed the diagnosis of epithelioid sarcoma. Cells stained positively for anti-vimentin, anti-cytokeratin and anti-epithelial membrane antigen, but not for anti-S100 protein and anti-actin antibodies. Wide local excision was performed. DISCUSSION: Epithelioid sarcoma is an uncommon malignant tumor which apparently differentiates from mesenchymatous cells. It is usually observed in the distal extremities in young males, predominantly the hands and forearms. The tumor presents a firm, flesh-colored indolent nodule. Ulceration usually develops and involves the subcutis and deeper soft tissue, particularly fascial planes, aponeuroses and tendon sheaths. Treatment is wide surgical excision with or without radiotherapy. The case reported here on the sole of the foot in a 78-year-old women is unusual. Clinicians should be aware that the initial biopsy may not be contributive and that repeated biopsies may be necessary for positive diagnosis.

Coexistent cutaneous T-cell lymphoma and B-cell malignancy. French Study Group on Cutaneous Lymphomas.

BACKGROUND: The coexistence of cutaneous T-cell lymphoma (CTCL) and a B-cell malignancy (BCM) is rare. OBJECTIVE: Our aim was to assess the clinical and pathologic aspects of coexistent CTCL and BCM and to examine potential explanations for this association. METHODS: We report six cases of concurrent CTCL and BCM in which B- and T-cell lineages were demonstrated by immunologic studies. The literature includes 13 additional cases. All 19 CTCL-BCM cases are reviewed. RESULTS: CTCL either preceded or followed the BCM, which was a low-grade malignancy in most cases (16 of 19). Possible explanations for the association include a genetic predisposition, underlying viral infection, chemotherapy-induced carcinogenesis, stimulation of a B-cell clone by malignant helper T cells, and alterations in progenitor cells before determination of B- and T-cell lineage. CONCLUSION: An alteration in progenitor cells, with subsequent oncogenic activation of variable origin, might account for most cases of coexistent CTCL and BCM.

[Metastatic basal cell carcinoma]. / Carcinome basocellulaire métastatique.

A case of basal cell carcinoma in a 17-year old male patient complicated, 5 years later, by inguinal and pulmonary metastases is reported. This clinical case raises two problems: the reality of the entity and the long-term follow-up of this type of tumours.