Cases reported "Carcinoma, Skin Appendage"

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1/55. 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. ( info)

2/55. Microcystic adnexal carcinoma: collaborative series review and update.

    BACKGROUND: Microcystic adnexal carcinoma (MAC) is a malignant appendageal tumor first described in 1982. It can be clinically and histologically confused with other malignant and benign cutaneous neoplasms, leading to inadequate initial treatment. This neoplasm is locally aggressive and deeply infiltrating, characterized by high morbidity and frequent recurrence. Mohs micrographic surgery has been used to conserve tissue and improve the likelihood for cure. OBJECTIVE: We report our experience using Mohs micrographic surgery for the treatment of MAC and compare with earlier reports in the literature. In addition, we review the epidemiology, clinical and histologic characteristics, and optimal treatment of this rare neoplasm. We also describe a 15-year-old white male patient with MAC on the scalp occurring only 7 years after radiation exposure. methods: The medical records of 11 patients with MAC who were treated by Mohs micrographic surgery were reviewed at both departments, and follow-up data were obtained. RESULTS: In all patients treated with Mohs micrographic surgery, there were no recurrences after a mean follow-up of 5 years. CONCLUSION: Mohs technique enables the detection of clinically unrecognizable tumor spread and perineural invasion often encountered with MAC. Aggressive initial treatment by microscopically controlled excision appears to offer the greatest likelihood of cure for this neoplasm, while providing conservation of normal tissue. In addition, we describe the second youngest patient with MAC and readdress the issue of previous radiotherapy as an important predisposing factor. ( info)

3/55. Metastatic microcystic adnexal carcinoma in an immunocompromised patient.

    BACKGROUND: Microcystic adnexal carcinoma is an uncommon, locally aggressive cutaneous neoplasm. To date, there are only two reports of histologically proven lymph node involvement with this tumor. We describe a case of a patient with microcystic adnexal carcinoma who developed multiple local metastasis in transit with histologically proven lymph node involvement and was diagnosed with chronic lymphocytic leukemia. OBJECTIVE: To describe the details of our case and to review what is currently known about this tumor. methods: Mohs micrographic surgery was utilized for tumor removal. RESULTS: This patient developed multiple tumors of the scalp over the period of a 1 year which were histologically proven to be microcystic adnexal carcinoma. All tumors were noncontiguous and presented on the scalp. During the histologic analysis of the last tumor removed by Mohs micrographic surgery a lymph node was resected which revealed infiltrative microcystic adnexal carcinoma. CONCLUSIONS: We present the case of an immunocompromised patient treated for microcystic adnexal carcinoma with Mohs micrographic surgery who proceeded to develop local metastasis in transit. ( info)

4/55. Microcystic adnexal carcinoma associated with primary immunodeficiency, recurrent diffuse herpes simplex virus infection, and cutaneous T-cell lymphoma.

    Cutaneous microcystic adnexal carcinoma (MAC) is a rare and poorly understood tumor that predominantly occurs in the head and neck. MAC usually affects people in their fourth and fifth decades. Some patients have had a history of radiation. We present a case of MAC occurring in the left antecubital fossa of an 18-year-old white woman with an unusual immunodeficiency syndrome. The patient also developed a squamous cell carcinoma, a cutaneous T-cell malignancy, and a perigastric leiomyoma. A congenital infection of herpes simplex virus (HSV) persisted throughout her life. The association of HSV infection with MAC and squamous cell carcinoma and that of peripheral T-cell lymphoma with Epstein-Barr virus is discussed in relation to her immunodeficiency. ( info)

5/55. Microcystic adnexal carcinoma: report of 13 cases and review of the literature.

    BACKGROUND: Microcystic adnexal carcinoma (MAC) is a rare tumor of the skin. Clinically it often masquerades as a firm, subcutaneous nodule on the head and neck regions. Microscopically it extends far beyond assessed clinical margins spreading locally in the dermal, subcutaneous, and perineural tissue planes. The local recurrence rate by standard excision is about 50%. Recent preliminary reports indicate more favorable cure rates with Mohs micrographic surgery (MMS). OBJECTIVE: To present our data on 13 cases (12 patients) of MAC treated by MMS. In addition, we reviewed the medical literature to summarize the accumulated experience of MMS treatment in the management of MAC. We also present a case of bilateral MAC of the face and describe a renal transplant recipient on immunosuppressive therapy who developed MAC of the nasal bridge. methods: We reviewed and updated our series of MAC cases treated by MMS over the last 9 years. A total of 13 cases of MAC are reviewed. We also searched the literature for MAC treated by MMS with a follow-up of more than 2-years. RESULTS: One patient had bilateral MAC of the nose and cheek. Another patient developed a MAC of the nasal bridge 20 years after renal transplantation. In this patient predisposing factors were radiation for teenage acne and immunosuppression therapy. A total of 13 cases of MAC were treated by MMS with no recurrences, with a mean follow-up of 5.0 years (range 1.1-8.0 years). CONCLUSION: We update the medical literature with 13 MAC cases treated by MMS. To our knowledge there have been 148 cases of MAC reported in the world literature. Including our series, there have been 73 cases of MAC treated with MMS. There were only four treatment failures. Regional and/or distant metastasis from MAC is rare, with only one reported death. Following MMS, the 2-year success rate was 89.7% (35 of 39). The accumulated data continue to confirm that when MAC is discovered early and is readily accessible to excision by MMS and other subspecialty support, a favorable outcome can be expected. ( info)

6/55. Neglected microcystic adnexal carcinoma: the second reported case in a black patient.

    BACKGROUND: Microcystic adnexal carcinoma (MAC) is a histologically aggressive appendageal neoplasm that often pursues a beguilingly indolent clinical course. MAC has previously been reported only once in a black patient. OBJECTIVE: To present the second MAC ever recorded in a black patient. methods: We describe a MAC of substantial dimension occurring in a black patient. The literature was subsequently examined for MAC occurring in ethnic patients. RESULTS: This MAC is remarkable not only for its occurrence in a black patient, but also because it is one of the largest MACs ever recorded (8 cm), it occurs in a relatively less common site (scalp), and its latency period before diagnosis is the longest ever definitively documented (31 years). CONCLUSION: MAC is rare among non-Caucasians. Its relatively indolent clinical growth, especially in sites that are poorly accessible to patient observation, may lead to significant delay in diagnosis and treatment. ( info)

7/55. Microcystic adnexal carcinoma: First reported case in an African American man.

    Microcystic adnexal carcinoma (MAC) is a relatively uncommon adnexal neoplasm that can display aggressive local invasion. MAC occurs most commonly on the central part of the face and can be clinically asymptomatic with a benign appearance. We describe the first reported case of MAC in an African American man who was treated by Mohs micrographic surgery. ( info)

8/55. 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. ( info)

9/55. Microcystic adnexal carcinoma: an uncommon tumor with debatable origin.

    BACKGROUND: Microcystic adnexal carcinoma is an uncommon skin appendage neoplasm exhibiting both pilar and sweat duct differentiation. This tumor remains a subject of controversy as to its differentiation profile, histogenesis, and classification which is reflected in the nomenclature used to designate the neoplasm in question. Beyond this controversy the tumor remains a diagnostic challenge because of its rarity, the histologic mimicry it may display, and its banal cytologic appearance; it also poses a therapeutic challenge, as it is characterized by slow but aggressive and destructive local growth extending beyond clinical margins together with a high tendency for perineural invasion and recurrence. OBJECTIVE: We report two cases of this unusual tumor illustrating some of its characteristics. Our review emphasizes the divergent opinions concerning its differentiation profile and its origin. An organoid nevus as the origin of microcystic adnexal carcinoma in one of our patients is discussed in this context. ( info)

10/55. Sebaceous carcinoma, trichoblastoma, and sebaceoma with features of trichoblastoma in nevus sebaceus.

    A 73-year-old woman had a linear yellowish plaque on the upper part of her right ear since birth. She presented because of the sudden growth of a nodule within the plaque. The plaque was waxy and yellowish, arching around the upper part of the ear. A reddish to yellowish large nodule was seen within the central part of the arc-shaped plaque; in addition, a small pigmented nodule, a small skin-colored nodule, and a few pigmented papules were observed in the anterior half of the arched plaque. Histopathologic examination revealed the large nodule to be sebaceous carcinoma, the small pigmented nodule to be trichoblastoma, the small skin-colored nodule to be sebaceoma with the features of trichoblastoma, a few pigmented papules to be superficial trichoblastomas due to primitive follicular induction, and the linear yellowish plaque to be nevus sebaceus. Although our literature search revealed scanty reports of definite cases of sebaceous carcinoma in nevus sebaceus, the presented case demonstrated the occurrence of sebaceous carcinoma in nevus sebaceus. Malignant neoplasms occurring in nevus sebaceous seem to be extremely rare, but care should be taken when a large nodule suddenly grows in a lesion of nevus sebaceus, especially in older adults. The presented case also suggested a close relation between trichoblastoma and sebaceoma. The cytokeratin staining pattern could not distinguish between sebaceous and follicular neoplasms in our case. ( info)
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