Malignant eccrine poroma of the hand.
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Eccrine porocarcinoma is a rare malignant tumor of the true sweat gland. It commonly presents in the lower extremities with lymphatic metastasis. The authors describe the clinical presentation, radiographic evidence, operative discoveries, and pathological findings in a patient with an eccrine porocarcinoma involving the soft tissue of the occiput, which had eroded through the cranium. A review of the literature failed to reveal any other such case. The discussion includes the epidemiology, pathogenesis, treatment, and outcome of eccrine porocarcinomas. The six reported cases of scalp eccrine tumors are reviewed.
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CONTEXT: Several aspects of sweat gland carcinomas (incidence, classification, diagnosis, and behavior) have not been definitively clarified and need to be studied further. OBJECTIVE: The clinicopathologic findings of a large series of sweat gland carcinomas, collected during a period of 15 years, are presented. METHODS: Sixty sweat gland carcinomas (41 porocarcinomas, 3 syringomatous carcinomas, 8 ductal carcinomas, 5 adenoid cystic carcinomas, and 3 mucinous carcinomas) were analyzed histologically and immunohistochemically. RESULTS: Porocarcinomas were composed of eosinophilic and clear atypical cells arranged in solid-cystic lobular masses. These tumors were divided into 2 subgroups: horizontal porocarcinomas, showing a prominent intraepidermal component, and nodular porocarcinomas, which demonstrated predominant nodular growth. Syringomatous carcinomas presented keratinizing and nonkeratinizing cysts, dilated tubules (sometimes with a "tadpole" appearance), small neoplastic ducts, solid islands, and cellular cords. Ductal carcinomas were characterized by a prominent formation of tubules, solid islands, and cellular cords. Adenoid cystic carcinomas presented a characteristic pattern, showing basaloid monomorphous cells with moderately atypical nuclei, arranged in cribriform or solid islands and in tubular structures. Mucinous carcinomas were composed of moderately atypical cells with eosinophilic vacuolated cytoplasm, forming solid and cystic islands floating in large mucin pools. Immunohistochemically, cytokeratin was found in neoplastic cells in all cases, carcinoembryonic antigen was detected in 73% of cases, and actin-positive (myoepithelial) cells were not found. CONCLUSIONS: Although numerous studies have been published in recent years, the histologic features, histogenesis, and classification of sweat gland carcinomas still remain controversial and need to be clarified by further studies.
BACKGROUND: Porocarcinoma is a rare eccrine sweat gland tumor, and a varied treatment is not yet determined for metastatic disease because of the chemo-resistant and radio-resistant nature of this tumor. CASE: This report describes a case of porocarcinoma arising on the penile shaft of 83-year-old man with extensive lymph node metastases. He was treated with emasculation and bilateral ilio-inguinal lymph node dissection. Histological examination of the resected tumor showed a homogeneous round cells with duct-like formation in some part and Pagetoid infiltration was also noted in the dermis. Immunohistochemical examination demonstrated a positive CEA and negative S-100 staining and this result was consistent with the pathological diagnosis of porocarcinoma. One course of chemotherapy which consisted of methotrexate, cisplatin, adriamycin, and bleomycin was given to the patient following surgery for the treatment of residual lymph nodes in the paraaortic area. Abdominal CT scan revealed partial response, about -94% shrinkage, after one course of chemotherapy. The duration of the response lasted four 4 months. He died of pneumonia caused by MRSA after 4 months and the autopsy revealed multiple liver metastases and a massive infiltration of tumor cells in the bone marrow of lumbar vertebra. CONCLUSIONS: There is no definite treatment modality available for metastatic porocarcinoma. Because of the patient's age, only one course of chemotherapy was given, however, a fairly good response against this rare tumor suggested that this new regimen might be effective against porocarcinoma.
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A 67-year-old patient was admitted for diabetes mellitus. Incidentally a large ulcerating tumour was found on the right upper leg. The patient had been suffering from the lesion for six years but had never dared to show it to her physician. Notwithstanding negative outcome of thin needle aspiration oncological dissection was carried out. Histopathological examination proved the tumour to be a malignant clear cell hidradenoma, which is a rare eccrine tumour. This tumour is known to be aggressive and easily spreads to locoregional lymph nodes. Even though it is a rare disease, the aggressive behaviour and the poor prognosis warrant alertness to this neoplasm.
A 75-year old woman without remarkable medical history presented with a large eccrine porocarcinoma of the trabelucar type located on her leg. This tumor was associated with two invasive squamous cell carcinomas of the legs, two Bowen's diseases of her thigh and arm and multiple actinic keratoses of the face. Despite major surgery, local recurrence of the porocarcinoma was observed 6 months later. During the 3-year follow-up after a second surgical intervention, this lesion did not reccur, but the patient developed a rapidly enlarging ulcerated tumor of the forehead which proved to be tricholemmal carcinoma. Although no carcinogenetic factor or familial history of multiple neoplasms could be evidenced, the occurrence of multiple tumors of various histogenesis over a 1 year period of time is unlikely to be fortuitous. Such an association of rare adnexal neoplasms has not yet been described.
We report a 58-year-old Chinese female who presented with a pigmented skin lesion on the right thigh, associated with a single ipsilateral inguinal lymph node without distant metastases detected. A wide excision of the tumour, split skin graft coverage and an en-bloc ipsilateral superficial groin dissection was performed. Histopathology revealed malignant skin adnexal tumour or porocarcinoma, with nodal metastases. She is presently at six months follow-up without any recurrence.
Clear cell hidradenoma was diagnosed in a girl at 3(1/2) years of age. Only one case of hidradenoma has been documented in the first decade of life, although it remains unclear whether it was clear cell hidradenoma. This case demonstrates that clear cell hidradenoma is a rare differential diagnosis of cutaneous tumors even in young children, which is of special interest, because malignant transformations can occur.
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Malignant eccrine poroma (MEP) is rare and both clinical and histologic diagnosis is often difficult. Therefore, diagnosis is sometimes delayed or even incorrect. We report a case in a 70-year old man with MEP of the leg. He demonstrated typical MEP behavior and the problems of differential diagnosis.
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BACKGROUND: Porocarcinoma is a malignant tumour of the eccrine sweat duct, arising from acrosyringium. The tumoral lesions involve the deep dermal tissue. CASE REPORT: We report the case of an 84 year-old woman, suffering from a porocarcinoma, extensively involving the major part of the left lower limb. The first symptoms appeared two years ago. Hundreds of metastatic papules and small nodules were present, isolated or confluent into large plaques. The clinical picture was very close to lymphangioma. Diagnosis was confirmed by histopathologic examination. Radiotherapy was useful short-term, allowing partial flattening of the lesions and improving lymphatic drainage, thus providing comfort for the patient. It did not prevent a later progression of the tumoral process. DISCUSSION: Porocarcinoma is a rare tumour that usually appears as a single nodule or a plaque, arising from a preexistent eccrine poroma, or developing de novo. Two histopathological variants are described: trabecular or epidermotropic. This latter form, observed in the present case, is more aggressive, leading to frequent local recurrences and/or metastases. Our report is exceptional: the literature shows only one other case with such widespread cutaneous involvement. The clinical course of our case is discussed.