Eccrine porocarcinoma of the vulva: a case report and review of the literature

Abstract

BackgroundMalignant tumors arising from the vulva account for only 0.6 % of all cancers in female patients. The predominant histologic type, representing about 90 % of these malignancies, is squamous cell carcinoma. Eccrine porocarcinoma is a rare malignant tumor arising from sweat glands. The incidence of eccrine porocarcinoma is estimated at 0.005–0.01 % of all cutaneous tumors. To the best of our knowledge, only seven previous cases of vulvar eccrine porocarcinoma have been reported in the English-language literature. We present the case of a patient with eccrine porocarcinoma of the vulva, and we summarize the clinical features of this disease using seven previously reported cases.Case presentationA 54-year-old Japanese woman visited a local hospital complaining of fever and left vulvar pain for 2 months. An initial examination revealed a 1 × 1 cm, firm, ulcerative mass in the inner aspect of the left labium minorum. With a preoperative diagnosis of vulvar squamous cell carcinoma, we performed a radical local excision followed by bilateral inguinal lymphadenectomy. Histological examination showed eccrine porocarcinoma, stage IB (T1bN0M0). Radiation therapy with weekly cisplatin administration was then given as adjuvant therapy. One month after treatment was completed, computed tomography revealed multiple metastases in the bilateral lungs and in the sacral bone. The patient received three courses of chemotherapy (paclitaxel and carboplatin) and underwent palliative radiation therapy to the sacrum. She died of her disease 12 months after surgery.ConclusionsWe report the case of a patient with eccrine porocarcinoma of the vulva and summarize the clinical features and the treatment options of eccrine porocarcinoma from a few retrospective case reports. Although eccrine porocarcinoma is a rare disease, clinicians and pathologists should be aware of its clinical and histological features and its biological behavior.

Porocarcinoma: a rare cause of lateral cervical tumor

“…The response of skin adnexal tumors to chemotherapy and/or radiotherapy may vary considerably from patient to patient. Patients can survive disease-free for many years after recurrence [ 23 , 24 ] or may conversely have a fulminant oncologic evolution from an apparently early-stage tumor, despite aggressive adjuvant treatment [ 25 ]. Our patient had a disease-free interval of 15 months after primary surgery of the skin lesion.…”

Sentinel lymph node biopsy in porocarcinoma: A case reports

“…Some authors suggested a possible association with radiation exposure and immunosuppression although an excessive sun exposure does not seem to be a significant risk factor [ 4 ]. EPC may arise de novo or can develop from a pre-existing benign lesion; some clinical signs, such as spontaneous bleeding, sudden growth and ulceration in a longstanding stable lesion must lead to the suspicion of malignant degeneration [ 10 ]. Clinically EPC can be presented as an erythematous or violaceous nodule, papule or plaque with an infiltrative or erosive pattern.…”

“…Therapeutic options for the treatment of EPC include electrofulguration, electrocautery, surgical excision, radiation and amputation. Surgical excision with histologically clear margins is generally considered the treatment of choice with cure rates as high as 70–80 %, although a recurrence rate of up to 20% has been reported [ 10 ]. This elevate incidence of local recurrence may be due to a not optimal free margin at surgical excision.…”

Cellular composition and microanatomy of lymphoid formations of the vestibule of the vagina in postnatal ontogenesis

“…Даже в современных монографиях и сводках по морфологии иммунной системы данный вопрос не рассматривается [1]. Это, с одной стороны, не способствует созданию целостной картины по вопросам структуры и функции иммунной системы в целом, с другой -не благоприятствует разработке новых схем профилактики и многочисленных заболеваний влагалища [2,3].…”