The occurrence of large cell transformation has been well documented in a subgroup of patients with mycosis fungoides/Sezary syndrome (MF/SS). However, because of the rarity of MF/SS, little is known about the influence of clinicopathologic features in predicting large cell transformation and about outcome in the transformed cases. We evaluated all patients with MF/SS who were registered in our clinic during the study period and for whom pathologic slides for review were available or could be obtained. Disease was classified as transformed if biopsy showed large cells (≥4 times the size of a small lymphocyte) in more than 25% of the infiltrate or if they formed microscopic nodules. Twenty-six patients with transformation were identified from a total of 115 evaluable cases with a diagnosis of MF/SS. The actuarial cumulative probability of transformation reached 39% in 12 years. The median time from diagnosis of MF/SS to transformation was 12 months (range, 0 to 128 months). Thirty-one percent of all patients with stage IIB-IV disease at presentation eventually transformed versus 14% of those with stage I-IIA (P= .03), with transformation being especially common in patients with tumors (T3), 46% of whom transformed. Combining elevated β2 microglobulin and lactic dehydrogenase (neither elevated v one or both elevated) was also predictive for transformation (P = .009). The median survival from initial diagnosis of MF/SS for the transformed patients was 37 months versus 163 months for the untransformed group (P = .0029). The median survival from transformation was 19.4 months (range, 2+ to 138 months). The following characteristics were associated with an inferior survival in transformed patients: (1) early transformation (<2 years from the diagnosis v ≥2 years; P = .011) and (2) advanced stage (IIB-IV v I-IIA; 2-year survival, 23% v 86%;P = .0035). We conclude that MF/SS patients with stages IIB-IV disease and, in particular, those with tumors have a high incidence of large-cell transformation. Patients with transformation have a relatively poor survival, especially if transformation occurs early (within 2 years) in the course of disease or if they are staged as IIB or higher. © 1998 by The American Society of Hematology.

The occurrence of large cell transformation has been well documented in a subgroup of patients with mycosis fungoides/Sezary syndrome (MF/SS). However, because of the rarity of MF/SS, little is known about the influence of clinicopathologic features in predicting large cell transformation and about outcome in the transformed cases. We evaluated all patients with MF/SS who were registered in our clinic during the study period and for whom pathologic slides for review were available or could be obtained. Disease was classified as transformed if biopsy showed large cells (≥4 times the size of a small lymphocyte) in more than 25% of the infiltrate or if they formed microscopic nodules. Twenty-six patients with transformation were identified from a total of 115 evaluable cases with a diagnosis of MF/SS. The actuarial cumulative probability of transformation reached 39% in 12 years. The median time from diagnosis of MF/SS to transformation was 12 months (range, 0 to 128 months). Thirty-one percent of all patients with stage IIB-IV disease at presentation eventually transformed versus 14% of those with stage I-IIA (P= .03), with transformation being especially common in patients with tumors (T3), 46% of whom transformed. Combining elevated β2 microglobulin and lactic dehydrogenase (neither elevated v one or both elevated) was also predictive for transformation (P = .009). The median survival from initial diagnosis of MF/SS for the transformed patients was 37 months versus 163 months for the untransformed group (P = .0029). The median survival from transformation was 19.4 months (range, 2+ to 138 months). The following characteristics were associated with an inferior survival in transformed patients: (1) early transformation (&lt;2 years from the diagnosis v ≥2 years; P = .011) and (2) advanced stage (IIB-IV v I-IIA; 2-year survival, 23% v 86%;P = .0035). We conclude that MF/SS patients with stages IIB-IV disease and, in particular, those with tumors have a high incidence of large-cell transformation. Patients with transformation have a relatively poor survival, especially if transformation occurs early (within 2 years) in the course of disease or if they are staged as IIB or higher. © 1998 by The American Society of Hematology.

Two cases of metastasizing cellular dermatofibroma (cutaneous fibrous histiocytoma) are presented. The first patient, an 18-year-old man, had a nodule excised from his right upper thigh. He developed three local recurrences at 1.5, 2, and 2.5 years and metastasis to inguinal lymph nodes. He underwent lung segmentectomies for metastases 1.5 and 4 years later and was alive with no evidence of tumor at latest follow-up, which was 15 months after the last surgery. The second patient, a 33-year-old man, had a nodule removed from his right posterior neck. The tumor recurred 3 months later and was reexcised. Right cervical lymph node metastases were excised at 7 and 8 years. A year later, a right cervical lymph node dissection yielded one positive node of 35, and multiple metastases were excised from the right lung. The patient was alive with lung metastases 6 years later, which was the latest follow-up. Grossly, both tumors were single 2 cm nonulcerated dermal-subcutaneous nodules. Histologically, they were characteristic of cellular dermatofibroma; they were composed of plump to spindled "fibrohistiocytic" cells arranged in a storiform pattern and had areas of hemorrhage, hemosiderin, and infiltration between dermal collagen bundles peripherally. Recurrences and metastases were histologically similar except that lung metastases were cystic. The alternative diagnosis of angiomatoid malignant fibrous histiocytoma was considered for these two cases but was excluded because the tumors were partly dermal, had a well-defined storiform pattern, and lacked large blood lakes, multinodularity, a fibrous pseudocapsule, and surrounding chronic inflammation. We conclude that dermatofibromas can rarely metastasize. Risk factors for metastasis may include relatively large size, high cellularity, and local recurrence. Judging from these two cases, metastasizing dermatofibromas behave in an indolent manner.

We report a case of a 28-year-old human immunodeficiency virus-positive man. He presented with confluent verrucous papules and nodules on his scrotum that were due to herpes simplex infection.

Background: Sebaceous gland neoplasms are rare tumors that are associated with visceral malignancies in patients with Muir^Torre syndrome (MTS).The majority of the MTS-associated tumors reveal mutations in DNA mismatch repair (MMR) genes (most often hMSH-2 and hMLH-1) and microsatellite instability.The sebaceous gland lesions in patients with MTS can often precede or occur concurrently with the visceral neoplasms.The early recognition of those lesions and their differentiation from sporadic sebaceous gland tumors are critical for proper patient management. Here we investigate the MMR gene expression in a variety of sebaceous gland tumors, with or without associated visceral malignancy. Methods: We studied the expressions of hMLH-1and hMSH-2 in 10 consecutive sebaceous hyperplasias,10 sebaceus nevi,12 sebaceous adenomas, seven sebaceous carcinomas and the adjacent normal sebaceous glands using immunohistochemistry and paraffin-embedded sections. Results: The normal sebaceous glands and the glands of all the sebaceus nevi were positive for hMLH-1and hMSH-2. Loss of hMSH-2 expression was found in 1/10 (10%) sebaceous hyperplasias, 3/12 (25.0%) sebaceous adenomas, and 2/7 (28.6%) sebaceous carcinomas. Loss of hMLH-1 expression was seen in1/10 (10%) hyperplasias, 3/12 (25.0%) adenomas, and1/7 (14.3%) carcinomas. No concurrent loss of both hMLH-1and hMSH-2 was observed. Loss of MMR (either hMLH-1or hMSH-2) was detected in 80% of the benign sebaceous lesions associated with malignancy. In comparison, only 23% of sebaceous lesions not associated with malignancy showed loss of MMR proteins. No loss of hMSH-2 protein was found in the visceral cancer in one patient with hMSH-2-negative sebaceous adenoma. Conclusions: Our results confirm the previous reports of alterations of mismatch repair genes in the sebaceous neoplasms of patients with MTS. However, we showed that those changes also occur early at the stage of sebaceous hyperplasia, even in the absence of a visceral malignancy.This indicates the importance of the abnormal DNA mismatch repair in the progression of this disease.