Sentinel lymph node melanoma metastases: Assessment of tumor burden for clinical prediction of outcome in the first Multicenter Selective Lymphadenectomy Trial (MSLT-I).

PURPOSE: As clinical management decisions in patients with Stage III melanoma have become more complex, precise pathologic characterization of sentinel lymph node (SLN) metastases has become critical to guide management. The extent of SLN involvement correlates with risk of adverse outcomes, but reported methods of disease quantification vary. We examined SLN metastases from patients participating in an international clinical trial and compared several methods of tumor burden quantification. METHODS: SLNs from 146 node-positive patients in the first Multicenter Selective Lymphadenectomy Trial (MSLT-I) were centrally-reviewed and characterized by number of tumor-positive nodes, percent nodal area tumor replacement, maximum dimension of largest metastasis, tumor penetrative depth, number of tumor foci, metastasis microanatomic location, and extracapsular extension. These data were analyzed for correlation with non-SLN metastasis and melanoma-specific survival (MSS). RESULTS: The median number of tumor-involved SLNs was 1. The median maximum metastasis dimension was 1.11 mm. Median SLN area involvement was 1.5%. Tumor burden measures were highly correlated with each other. Factors associated with non-SLN metastasis by univariable analysis were primary tumor ulceration and extent of metastases. Tumor thickness, ulceration, non-SLN metastasis and multiple measures of SLN tumor burden were significantly related to MSS on univariable analysis. After multivariable adjustment, number of involved SLNs (p = 0.05) and percent nodal area tumor replacement (p = 0.02) were independent predictors of MSS. CONCLUSION: Central review of MSLT-I pathology indicates that primary tumor and SLN tumor characteristics predict non-SLN metastasis and MSS. Percent nodal involvement was more powerfully prognostic than the more commonly used maximum dimension of largest metastasis.

Indeterminant Cell Histiocytosis in a Patient With Hemophagocytic Lymphohistiocytosis.

ABSTRACT: Indeterminant cell histiocytosis is a rare disorder, which often presents as a benign, papular eruption without age or sex predilection. It is diagnosed based on histologic findings that are similar to those seen in Langerhans cell histiocytosis without the characteristic Birbeck granules. Hemophagocytic lymphohistiocytosis is a potentially life-threatening disease of immune overactivation that may present with severe symptoms and multiorgan involvement. It can develop because of a primary genetic mutation or secondary to a trigger. Despite their widely different clinical manifestations, both diseases originate from the monocyte-macrophage cell lineage. In this article, we describe the unique case of a 2-year-old patient presenting with both of these histiocytic pathologies.

Genital nevus lipomatosus cutaneous superficialis: A diagnostic challenge in a pediatric patient.

Nevus lipomatosus cutaneous superficialis (NLCS) is an idiopathic hamartomatous condition characterized by the presence of mature adipose tissue in the dermis. We report a case of NLCS initially misdiagnosed as condyloma acuminata in a 14-year-old boy. This case highlights classic clinical and histologic features of NLCS. The case presented here underscores the need for a high degree of clinical suspicion in diagnosing NLCS and in differentiating benign anogenital lesions from sexually transmitted conditions to avoid unnecessary work-up and undue emotional stress.

Bullous acral eruption related to secukinumab.

Skin reactions related to secukinumab are uncommon. Although the initial phase 3 studies of this medication reported infection and urticaria as adverse cutaneous events, other cases of unique adverse events have since been reported. We are presenting a patient who developed an exuberant hand and foot reaction after starting secukinumab.

Clear-cell atypical fibroxanthoma: An unusual case in an unusual place.

Atypical fibroxanthoma represents a low-grade sarcoma that usually presents in the elderly population on sun-damaged areas of the head and neck regions. This neoplasm is characterized on histology by atypical pleomorphic, epithelioid to spindled cells, arranged in a haphazard pattern, set within a background of solar elastosis. In this case report, we present a unique case of the rare variant of clear-cell atypical fibroxanthoma arising in an unusual place, specifically the lower extremities.

Lichenoid Dermatitis in an Adult with Immune Dysregulation, Polyendocrinopathy, Enteropathy, X-Linked (IPEX) Syndrome.

A 23-year-old man presented to our practice with erythroderma and an unusual retiform eruption, along with alopecia universalis and nail dystrophy. He had had no skin findings at birth, but since early infancy had had localized eczematous eruptions of his skin. At 10 years of age, he had developed a generalized eczematous flare requiring hospitalization, and another generalized episode occurred in October 2010. He was prescribed prednisone 60 mg daily, which initially provided an improvement, but tapering of the corticosteroid resulted in another generalized flare.

Dermal Mucinosis in Chronic Sclerodermoid Graft-Versus-Host Disease.

Dermal mucinosis is characterized by the deposition of glycosaminoglycans (mucin), either focally or diffusely within the dermis. This may occur as a primary idiopathic disorder or secondary to several dermatoses, most notably lupus erythematous, scleroderma, and dermatomyositis. The authors present an unusual finding of dermal mucinosis in association with chronic sclerodermoid graft-versus-host disease.

What Is the Best Method for Removing Biopsy-Proven Atypical Nevi? A Comparison of Margin Clearance Rates Between Reshave and Full-Thickness Surgical Excisions.

BACKGROUND: It is a common practice to biopsy clinically atypical nevi, which may signify an individual's increased risk of developing melanoma. There is no consensus in the current literature, however, as to what the best method is to manage biopsy-proven atypical nevi. OBJECTIVE: The objective was to compare margin clearance rates between reshave excision and full-thickness surgical excision performed to manage biopsy-proven atypical nevi. MATERIALS AND METHODS: In this retrospective observational study, histopathology specimens from 201 patients whose atypical nevi were surgically removed were analyzed. RESULTS: For the majority (76%-79%) of the atypical nevi studied, all atypical melanocytes were removed by the initial shave biopsy. Of those atypical nevi with positive margins, shave re-excision was shown to have a lower clearance rate (76.2%) when compared with surgical excision (87.5%). CONCLUSION: This study shows that in most cases, no residual atypical melanocytes are left after the initial shave biopsy. However, of the lesions where margins are not clear, full-thickness surgical excision may have a higher rate of success at eventual clearance than reshave excision.

Perforating lichen nitidus.

Transepidermal elimination and/or perforation of the epidermis is seen in a variety of conditions but has only rarely been reported in association with lichen nitidus. We describe a case of lichen nitidus with the unique finding of epidermal perforation, a feature that made the usual straightforward diagnosis of lichen nitidus much more difficult.

An unusual case of porocarcinoma arising on the scalp of a 22-year-old woman.

Porocarcinoma is a rare malignant neoplasm that arises from the intraepidermal ductal portion of the eccrine sweat glands. This neoplasm most often presents in elderly individuals on the lower extremities. We present an unusual case of porocarcinoma arising on the scalp of a young patient. To the best of our knowledge, there have been fewer than 20 cases of porocarcinomas arising at this site reported in the literature.

A rare case of melanoma with Touton-like giant cells: a potential diagnostic pitfall.

Although approximately 876,000 individuals in the United States currently have a diagnosis of melanoma, the Touton-like giant cell variant has been described only twice in the literature to date. In our case, a 70-year-old man with a history of sclerosing carcinoma on the scalp presented for evaluation of a new nodularity at the site of his previous surgery. On examination, a new complex pigmented lesion on the posterolateral scalp, adjacent to the recurrent sclerosing carcinoma, was noted. Biopsy of the pigmented lesion revealed an invasive melanoma with a Breslow depth of at least 2.78 mm. Microscopic sections showed a predominantly dermal-based tumor composed of sheets and nests of enlarged epithelioid cells. These cells contained oval-to-reniform nuclei with prominent nucleoli and an abundant amount of eosinophilic to vacuolated cytoplasm. Interestingly, numerous multinucleated melanocytes, some with a "Touton" appearance, were scattered throughout the lesion. The lesional cells demonstrated positivity to Mart-1 and HMB-45. Fortunately, the patient's sentinel lymph node biopsy was negative for micrometastases, and a subsequent Position Emission Tomography (PET) scan was unremarkable. Documentation of individual cases of this rare histologic variant of melanoma is necessary given the ability of this lesion to mimic benign histiocytic proliferations at scanning magnification.

Cutaneous granulomas and epidermodysplasia verruciformis in early onset combined immunodeficiency syndrome.

Cutaneous granulomas with prominent caseating necrosis are a rare manifestation of immunodeficiency. Extensive and recalcitrant cutaneous viral infections can also be seen. We present a case of an 18-year-old white man with an early onset poorly characterized combined immunodeficiency syndrome who, over the past 5 years, developed enlarging tender red-purple plaques on his extremities and pink near-confluent macules on his chest and back. Previous biopsies of the red-purple plaques showed features of granuloma annulare. Histopathological examination of old and new biopsies revealed both sarcoidal and palisading necrobiotic granulomas with perforating features and elastophagocytosis. Stains and tissue cultures were negative for bacterial and fungal organisms. In addition, biopsy of a macule on the back demonstrated verruca plana with characteristics of epidermodysplasia verruciformis. As an infant, the patient had failure to thrive and a combined immunodeficiency, but was lost to follow-up for 15 years. He currently continues to have severe hypogammaglobinemia and cellular immunodeficiency. Intravenous immunoglobulin and prednisone were initiated and his plaques improved rapidly. Topical imiquimod was ineffective for the verruca plana. The patient and his parents are currently undergoing whole exome sequencing including evaluation for epidermodysplasia verruciformis 1 and 2 gene mutations. This case highlights the importance of including genetic immunodeficiency disorders in the clinical and histopathological differential diagnosis for cutaneous sarcoidal or palisading necrobiotic granulomas and for extensive cutaneous viral infection.

Atypical fibrous histiocytoma arising in the perianal area: a case report and review of the literature.

Atypical fibrous histiocytoma (AFH) is an uncommon variant of cutaneous fibrous histiocytoma that can display histologic features associated with malignancy. Fewer than 150 cases have been reported in the literature. The majority of these lesions present on the trunk and extremities of middle-aged women. Genital lesions are rare, with one documented case of vulvar AFH and another case of scrotal AFH in the literature. We report an additional case of a 68-year-old woman who was diagnosed with an AFH in an unusual location, the perianal area. Histologically, the lesion was characterized by a nodular fairly well-circumscribed proliferation of large epithelioid macrophages with scattered lymphocytes and mast cells in the background. The atypical macrophages contained enlarged markedly pleomorphic nuclei with prominent nucleoli. Scattered multinucleated "monster cells" and atypical mitoses were observed throughout the lesion. Immunologically, the lesional cells were focally positive when stained with antibodies against CD163 and Factor XIIIa. They were negative for CD34, CD31, desmin, smooth muscle actin, CAM 5.2, keratin 5/6, S100, CD3, CD20, and CD30. The constellation of histologic and immunologic features was most consistent with an AFH. To our knowledge, this case is the first perianal presentation of AFH to date.

An unusual presentation and distribution of generalized eruptive syringomas.

A 19-year-old Caucasian man presented with numerous erythematous to flesh-colored papules that appeared in crops on his neck, axillae, buttocks, and lower back. The lesions started on his anterior neck at age 12. At 18 years, new crops of papules appeared on his axillae, back, and buttocks over several months. He reported pruritus in the lesions following exercise and perspiration. He denied any family history of similar lesions. His primary care physician treated him with topical triamcinolone 0.1% cream, which made the lesions smaller, less erythematous, and less pruritic; however, the papules never fully resolved. After discontinuation of the steroids, these erythematous pruritic papules gradually recurred in the same areas of his body. The patient denied any other medical complaints.