Cases reported "Carcinoma, Skin Appendage"

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1/12. Microcystic adnexal carcinoma arising in the setting of previous radiation therapy.

    While there are several reports of microcystic adnexal carcinoma developing in patients within sites of previous therapeutic irradiation, this relationship is not well described in the dermatologic literature. We report a case of a 42-year-old man with a remote history of therapeutic irradiation following surgical resection of periorbital rhabdomyosarcoma. Subsequently, he developed multiple basal cell carcinomas and a microcystic adnexal carcinoma within the field of irradiation. The histologic features were those of a classic microcystic adnexal carcinoma, with well differentiated nests and cords of keratinocytes displaying follicular and ductular differentiation infiltrating diffusely into the reticular dermis. Dense fibrosis was present surrounding the neoplastic keratinocytes. Nuclear atypia and mitotic figures were not identified. A carcinoembryonic antigen (CEA) stain demonstrated glandular differentiation. It is important for dermatologists to be aware of the apparent relationship between the rare microcystic adnexal carcinoma with its innocuous scar-like clinical appearance and prior local radiation therapy.
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2/12. Microcystic adnexal carcinoma of the vulva.

    BACKGROUND: Microcystic adnexal carcinoma (MAC) is a subset of sweat gland carcinoma first described as a specific entity by D. J. Goldstein, R. J. Barr, and D. J. Santa Cruz (Cancer 1982;50:566-72). We report the first case of MAC occurring on the vulva and review the literature pertaining to this rare tumor. CASE: A 43-year-old multiparous black woman presented initially to Kings County Hospital Medical Center with a chief complaint of a vulvar lesion arising on the left labia majora which she had noted for 4 years prior to presentation. Aside from increasing paresthesia in the area, she denied any constitutional symptoms. Her past medical history was significant only for hyperthyroidism and mild hypertension and surgical history was noncontributory. Gynecologic history was unremarkable, with sporadic care over the last 20 years. physical examination revealed a 1.5 x 2.0-cm raised, well-circumscribed, firm mobile lesion on the left labia majora. It was noted to be yellow in color with the surrounding tissue being unremarkable in character. The remainder of her gynecologic examination and lymph node survey was unremarkable. Preoperative chest X ray was negative as was the CAT scan of the abdomen and pelvis. All laboratory values were within normal limits. A Pap smear done preoperatively was significant for atypical squamous and glandular cells of undetermined significance. Subsequent colposcopic examination of the cervix was remarkable for cervicitis and was adequate, with the entire transformation zone visualized. Both endocervical curettage and endometrial biopsy were normal. Initially, an excisional biopsy was performed with final pathology demonstrating microcystic adnexal carcinoma with positive surgical margins. She subsequently underwent a left radical hemivulvectomy with bilateral inguinal groin lymph node dissection. At the time of surgery, the left labia majora was noted to be well healed, with a residual surgical scar easily discernible. No areas of discoloration were noted and digital palpation of the area was unremarkable. Microscopic residual tumor was noted; however, all surgical margins and lymph nodes were negative for tumor. Her postoperative course was unremarkable. The patient has continued to do well since the time of her surgery and is being followed conservatively. CONCLUSION: Radical vulvectomy should be performed when MAC occurs in the vulva to secure negative margins of resection. groin dissection should be reserved for cases in which the inguinal lymph nodes are clinically suspicious or in cases of tumor recurrence.
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3/12. Recurrent polymorphous sweat gland carcinoma of the skin.

    Polymorphous sweat gland carcinoma is an unusual, recently described variant of low-grade malignant adnexal neoplasm of the skin characterized by a prolonged clinical course and predilection for the extremities. We describe a case of recurrent polymorphous sweat gland carcinoma in a 56-year-old man who presented with multiple large skin nodules distributed along the flexor surface of his left arm. The lesions were treated by surgical excision; multiple local recurrences, as well as the development of new lesions, were observed over a period of 5 years. No distant metastases have been observed so far. The clinical differential diagnosis and management of these unusual lesions are discussed.
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4/12. Microcystic adnexal carcinoma of the breast: a very rare breast skin tumor.

    BACKGROUND: Microcystic adnexal carcinoma is a rare, slowly progressing, malignant tumor of sweat gland origin. OBJECTIVE: A case of microcystic adnexal carcinoma of the breast with a history of 20 years is presented. methods: On initial examination, the ulcerative cutaneous mass was fixed to the underlying breast tissue and chest wall. Both the macroscopic appearance of the tumor and its location suggested a glandular breast carcinoma. However, an incisional biopsy revealed the nature of the tumor as microcystic adnexal carcinoma of the breast skin. Following the diagnosis, a wide excision of the tumor was carried out for the final treatment. RESULTS: To our knowledge this is the first case of microcystic adnexal carcinoma arising in the breast skin presented in the literature. CONCLUSION: This case demonstrates that microcystic adnexal carcinoma can occur on the breast skin and should be treated with wide excision due to its locally aggressive behavior.
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5/12. Sclerosing sweat duct carcinoma of the orbit.

    Sclerosing sweat duct carcinoma or microcystic adnexal carcinoma is a rare, aggressive and locally destructive sweat gland malignancy. It is difficult to diagnose clinically and histologically and as such is probably under-recognized. Late recognition is common and is associated with extensive tissue loss. Local extension into the orbit from periorbital cutaneous lesions is documented, necessitating exenteration for adequate tumor clearance. We report a case of orbital sclerosing sweat duct carcinoma without a clinically evident cutaneous lesion, which required exenteration in a patient with no identifiable risk factors. Inclusion of sclerosing sweat duct carcinoma in the clinical and histological differential diagnosis of orbital masses and reporting of cases will lead to increased recognition of this malignancy, add to our understanding of its natural history and improve patient outcomes.
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6/12. Spiradenocarcinoma of the breast arising in a long-standing spiradenoma.

    The embryologic origin of the breast is related to salivary and sweats glands. Thus, breast neoplasms may show differentiation toward these tissues, although this is a rare event in humans. We report the clinicopathologic and immunohistochemical features of a 57-year-old woman presenting with a spiradenoma that originated in breast tissue and became malignant 40 years later. Some histogenetic concepts relevant to this case are discussed, along with a brief review of this neoplasm.
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7/12. Microcystic adnexal carcinoma: report of four cases treated with Mohs' micrographic surgical technique.

    BACKGROUND: Microcystic adnexal carcinoma (MAC) is a rare and aggressive malignant tumor of the sweat glands. Clinically, it often presents as a firm subcutaneous nodule on the head and neck regions. On histology, MAC exhibits both pilar and sweat duct differentiation with a stroma of dense collagen. It often extends beyond the clinical margins with local spreading in the dermal, subcutaneous, and perineural tissue planes. It has a high local recurrence rate after standard excision. Recent preliminary reports have indicated more favorable cure rates with Mohs' micrographic surgery (MMS). OBJECTIVE: To present our data on four cases of MAC treated by MMS. We also compared our findings with more recently reported series in the English language literature. methods: We reviewed the medical records of four patients (two males and two females) with MAC treated by MMS over the last 3 years. We also obtained follow-up data. RESULTS: In all four patients with MAC treated by MMS, there were no recurrences, with a mean follow-up of 1 year. CONCLUSION: We report an additional four MAC cases treated by MMS. The accumulated data continue to confirm that, if the diagnosis of MAC is made early, and if the anatomic location is accessible to excision by MMS, a favorable outcome can be expected.
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8/12. Microcystic adnexal carcinoma. Ten cases treated by Mohs micrographic surgery.

    BACKGROUND. Microcystic adnexal carcinoma is a slow-growing, nondescript, locally aggressive, deeply infiltrating neoplasm histologically characterized by an infiltrative pattern of basaloid or squamous cells, a desmoplastic stromal reaction, keratin-filled cysts, and glandular structures. OBJECTIVE. Microcystic adnexal carcinoma is uncommon and may be mistaken microscopically for other benign and malignant entities. Perineural or intraneural involvement by tumor cells is characteristic and extension into underlying structures including muscle, fat, and bone are frequently encountered. Although local recurrences are common after standard surgical excision, metastases have not been reported. Extensive resections of lesions may be necessary to extirpate widespread tumor, particularly those that are long standing or recurrent. Because significantly increased morbidity is associated with recurrent disease, surgical and histopathologic techniques that stress examination of all margins are advantageous. methods. We review the course of 10 patients with microcystic adnexal carcinoma of the face (six primary and four recurrent lesions) and their treatment by Mohs micrographic surgery.
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9/12. Microcystic adnexal carcinoma (sclerosing sweat duct carcinoma).

    Microcystic adnexal carcinoma is a relatively uncommon tumor first described in 1982. Most commonly presenting as a facial plaque or nodule, this highly invasive tumor is characterized by slow growth and relentless local destruction. We report the presence of microcystic adnexal carcinoma in the neck, a previously unreported site. The patient was a 48-year-old man who had received radiation therapy at age 5 to shrink the thymus gland and subsequent radiation therapy after excision of a thyroid tumor at age 35. The results of immunohistochemical stains were consistent with the hypothesis that this tumor develops from pluripotential cells capable of follicular and eccrine differentiation.
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10/12. A tumour derived from Ebner's glands: microcystic adnexal carcinoma of the tongue.

    Microcystic adnexal carcinoma (MAC) is known as an infiltrating but non-metastasizing tumour of the skin, that derives from sweat glands or follicular epithelium. We report on a rare case of MAC of the tongue. The patient had noticed the tumour for two years with slowly increasing dysphagia but no other symptoms of an oropharynx carcinoma. Histological and immunohistochemical analyses showed a similarity between the tumour derived from Ebner's glands of the tongue and MAC of the sweat glands.
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