Answer of Dermatopathology Case 96

Immunohistochemistry: CD30 positive cells
[ CD3 & CD4 are faintly positive ; ALK and EMA -Negative]

Primary Cutaneous Anaplastic Large Cell Lymphoma

Abstracts:

Primary cutaneous anaplastic large-cell lymphoma.Dermatol Online J. 2010 15;16(11):2.

Primary cutaneous anaplastic large-cell lymphoma (ALCL) is a form of cutaneous T-cell lymphoma that is characterized by solitary or localized nodules or plaques. Histopathologic features include a diffuse, non-epidermotropic infiltrate with cohesive sheets of large anaplastic CD30+ tumor cells. This entity must be distinguished from systemic ALCL with cutaneous involvement and lymphomatoid papulosis. Treatment modalities include clinical monitoring, radiation therapy, and surgical excision, with systemic chemotherapy reserved for disseminated or extracutaneous disease.

CD30(+) anaplastic large cell lymphoma: a review of its histopathologic, genetic, and clinical features. Blood.2000 Dec 1;96(12):3681-95.

Anaplastic large cell lymphoma (ALCL) represents a generally recognized group of large cell lymphomas. Defining features consist of a proliferation of predominantly large lymphoid cells with strong expression of the cytokine receptor CD30 and a characteristic growth pattern. With the use of molecular and clinical criteria, 3 entities of ALCL have been identified: primary systemic anaplastic lymphoma kinase (ALK)(+) ALCL, primary systemic ALK(-) ALCL, and primary cutaneous ALCL. ALK expression is caused by chromosomal translocations, most commonly t(2;5). ALK(+) ALCL predominantly affects young male patients and, if treated with chemotherapy, has a favorable prognosis. It shows a broad morphologic spectrum, with the "common type," the small cell variant, and the lymphohistiocytic variant being most commonly observed. The knowledge of the existence of these variants is essential in establishing a correct diagnosis. ALK(-) ALCL occurs in older patients, affecting both genders equally and having an unfavorable prognosis. The morphology and the immunophenotype of primary cutaneous ALCL show an overlap with that of lymphomatoid papulosis. Both diseases have an excellent prognosis, and secondary systemic dissemination is only rarely observed. The described ALCL entities usually derive from cytotoxic T cells. In contrast, large B-cell lymphomas with anaplastic morphology are believed to represent not a separate entity but a morphologic variant of diffuse large B-cell lymphoma. Malignant lymphomas with morphologic features of both Hodgkin disease and ALCL have formerly been classified as Hodgkin-like ALCL. Recent immunohistologic studies, however, suggest that ALCLs Hodgkin-like represent either cases of tumor cell-rich classic Hodgkin disease or (less commonly) ALK(+) ALCL or ALK(-) ALCL.

Primary cutaneous anaplastic CD30(+) large-cell lymphoma that completely regressed after incisional skin biopsy. Cutan Ocul Toxicol. 2010 Nov 18.

We describe a 48-year-old woman with three erythematous nodules localized on the left forearm, with 2 months evolution. Histological and immunohistochemical examination revealed a CD30(+) large-cell lymphoma. Systemic involvement was not detected. The tumor regressed spontaneously within a week, after the incisional skin biopsy. In control skin biopsy, there was not any histological feature of lymphoma. No reactivation or any symptom of systemic disease was observed during the 10-month follow-up period.

Cutaneous anaplastic large cell lymphoma: clinicopathologic, immunohistochemical and prognostic study of 44 cases. Zhonghua Bing Li Xue Za Zhi. 2010 Apr;39(4):230-4.

OBJECTIVE: To study the clinicopathologic features, immunophenotype and prognosis of primary cutaneous anaplastic large cell lymphoma (CALCL).
METHODS: Histopathologic evaluation and immunohistochemical study by Envision method were carried out in 44 archival cases of CALCL. The clinical information and follow-up data were analyzed.
RESULTS: The patients presented with skin nodules, masses or plaques, sometimes associated with ulceration. The commonest sites of involvement were the extremities. Follow-up data were available in 39 patients. The overall survival rate was 87.2% (34/39). Disease relapses were detected in 46.2% (18/39) of the patients. Statistical analysis indicated that patients older than 50 years of age or with no less than two involved anatomic sites were more likely to have disease relapses.

Primary cutaneous anaplastic large cell lymphoma: clinical presentation, therapy and prognosis study of 10 cases.Zhonghua Yi Xue Za Zhi. 2010 May 11;90(18):1247-50.

OBJECTIVE: To explore the clinical presentation, therapy and prognosis study of primary cutaneous anaplastic large cell lymphoma (PCALCL).
METHODS: We reviewed and analyzed ten cases of PCALCL receiving treatment at our hospital from January 1999 to January 2009.
RESULTS: There were 8 males and 2 females with a median age of 48 years old (range: 22 - 69). There were single subcutaneous nodule (n = 7) and multiple nodules (n = 3). And the lesions could be found on head and neck (n = 5), trunk (n = 3) and all over body (n = 2). The lesions appeared red, solid and stable subcutaneous nodules. Partial lesions had a spontaneous regression and new nodules appeared at the same or different sites. Two patients had lymphadenopathy and one had bone involvement with anaplastic lymphoma kinase (ALK) positive and high cell proliferation ratio index (ki-67 > 80%). Seven cases with single lesion received surgical excision plus radiotherapy, chemotherapy or radiochemotherapy, one case recurred, six cases survived without disease. Three cases with multiple lesions received systemic chemotherapy mainly in combination with radiotherapy or biotherapy, two cases recurred and one case survived without disease. The median follow-up was 44 months (range: 9 - 95), progression free survival 89% and overall survival 100%.
CONCLUSION: PCALCL is found more commonly in males. Visceral and lymph node involvement are rare. The patients with single lesion have a longer disease-free survival than those with multiple lesions after surgical excision in combination with chemotherapy or radiotherapy. Multiple lesions can not be cured.