Primary cutaneous CD30+ anaplastic large cell lymphoma: report of a rare case

Abstract

Primary cutaneous anaplastic large cell lymphoma (PCALCL) is a rare type of non-Hodgkin's lymphoma comprising approximately 0.9-9.0% of all cutaneous lymphomas. PCALCL is characterized by the absence of systemic involvement, spontaneous regression and low recurrence rate especially in localized lesions.We present a 47-year-old female with a 1½-year history of two asymptomatic erythematous indurated plaques on the right arm. Skin punch biopsy revealed dense infiltrates of non-epidermotropic, large, irregularly-shaped lymphocytes with hyperchromatic and pyknotic nuclei. Immunohistochemistry revealed that these atypical cells are anaplastic lymphoma kinase (ALK) positive, CD30+, CD3-, CD20-and epithelial membrane antigen (EMA) negative. Clinical, histopathological and immunohistochemical findings are consistent with PCALCL. Work-ups revealed no systemic involvement. Short course CHOP (Cyclophosphamide, Doxorubicin, Vincristine, and Prednisone) chemotherapy resulted in total resolution of skin lesions; however, recurrence was noted 12 months after treatment. She then underwent radiotherapy and achieved complete remission.Because the clinical presentation of PCALCL can be variable, a high index of suspicion is necessary in patients presenting with chronic plaques and nodules unresponsive to topical or oral medications.

ALK‐positive primary cutaneous anaplastic large cell lymphoma: a case report and review of the literature

“…Following the diagnosis of ALK‐positive pcALCL in the presented case, and in a pediatric patient that had been recently reported by our institution, we searched the literature for ALK‐positive pcALCL cases to better characterize the course and prognosis of this rare variant. Including our cases, we identified nine pediatric patients and 12 adults who were diagnosed with ALK‐positive pcALCL (Table ). Complete staging was performed in 15 of 21 cases.…”

“…pcALCL is generally found to be ALK-negative, and ALK expression in skin lesions of ALCL evokes high suspicion for secondary skin involvement by sALCL. 1 Recently, ALKpositive pcALCL has been reported in both children and adults [2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18] with heterogenous courses described.…”

Primary cutaneous anaplastic large-cell lymphoma in a patient with Hodgkin’s disease – case report

“…W takich przypadkach należy zawsze wykluczyć układową postać chłoniaka anaplastycznego wielkokomórkowego, przerzuty do skóry ziarnicy złośliwej oraz transformację CD30+ ziarniniaka grzybiastego, co w przeciwieństwie do C-ALCL wiąże się z gorszym rokowaniem i koniecznością wdrożenia agresywnego leczenia [6]. Rozpoznanie ustala się na podstawie obrazu histopatologicznego oraz immunohistochemicznego, potwierdzającego ekspresję antygenu CD30+ na ponad 75% komórek neoplazmatycznych, bez objawów LyP, ziarniniaka grzybiastego lub innego typu CTCL rozpoznanego przed diagnozą C-ALCL [7]. Pomimo niepokojącego obrazu histopatologicznego, sugerującego złośliwy charakter zmian skórnych, cechującego się obecnością anaplastycznych komórek nowotworowych z dużym atypowym jądrem, rokowanie jest bardzo dobre, a 5-letnie przeżycie osiąga 90% pacjentów.…”

Pyogenic variant of primary cutaneous CD30+ anaplastic large‐cell lymphoma: Spontaneous remission in a young adult

“…According to the 2005 WHO-EORTC classifi cation, CLPDs include lymphomatoid papulosis and primary cutaneous anaplastic large-cell lymphoma. In addition, "borderline" cases exist that exhibit features of both of the aforementioned entities [1][2][3] . In the literature, there have also been cases of a pyogenic lymphoma variant clinically characterized by purulent, fast-growing tumors -similar to an abscess or furuncle -and histologically marked by a particularly prominent neutrophilic infi ltration extending into the subcutis [4][5][6][7][8] .…”

“…The clinical fi ndings suggest an aggressive disease course. On the other hand, the condition has an excellent prognosis, with a 5-year survival rate of more than 90 % [ 1,[4][5][6] . Thus, the prognosis of the pyogenic subtype is by no means worse than that of other CLPDs [ 2 ] .…”