CD30-positive cutaneous lymphoma: report of four cases with an emphasis on clinicopathological correlations

Abstract: The classification of cutaneous lymphomas is multidisciplinary and requires the correlation between clinical, histopathological, immunohistochemical, and molecular diagnostic elements. In this article, we present four different cases of CD30-positive T-cell lymphoma with cutaneous manifestations. We compare cases with definitive diagnosis of papulosis lymphomatoid type C, primary cutaneous anaplastic large T-cell lymphoma, systemic anaplastic large T-cell lymphoma with secondary skin involvement, and mycosis fungoides with large cell transformation, highlighting the importance of clinicopathological correlation to classify these cases.

Primary cutaneous anaplastic large cell lymphoma with lung metastasis

Primary cutaneous anaplastic large cell lymphoma (PC-ALCL) is relatively a rare tumour of head and neck region. Without histopathological confirmation, it appears and behaved as like malignant epithelial tumor of the head and neck region as will be illustrated in the present case. Cutaneous involvement of the tumor with distant metastasis has made the prognosis unfavorable. This isolated case of head and neck PC-ALCL was highlighted as it has a tendency to behave aggressively and early diagnosis and treatment is crucial to avoid poor survival outcome.

Linfoma anaplástico de células grandes CD30 positivo cutáneo primario: reporte de un caso / Primary cutaneous anaplastic large cell lymphoma: Report of one case

Primary cutaneous anaplastic large cell lymphoma (PCALCL) is within the spectrum of cutaneous CD30-positive lymphoproliferative disorders. It presents as localized or multifocal tumors or plaques and carries an excellent long-term prognosis even in cases with regional and/or ipsilateral lymph node involvement or in cases of recurrent disease. We report a 34 year-old female with a thigh lesion. Skin biopsy confirmed the diagnosis of PCALCL. The patient was strictly monitored but no treatment was instituted and the tumor regressed spontaneously. After 24 months of follow-up the patient remains free of disease without new lesions.

A Case of Primary Cutaneous CD30+ Anaplastic Large Cell Lymphoma, Small Cell Variant / 대한피부과학회지

Primary cutaneous anaplastic large cell lymphoma (PCALCL) is a rare cutaneous T-cell lymphoma. Most cases are composed of large anaplastic cells. However, it presents a wide spectrum of histologic patterns. In the small cell variant, a small-sized pleomorphic cell morphology can be seen. A 74-year-old woman presented with an 8-month history of asymptomatic ulcerative plaque and satellite nodule on the right calf. Her past medical history was not specific. The histologic findings on punch biopsy specimens showed a malignant small round cell tumor on both lesions. The tumor cells had large pleomorphic nuclei with multinucleation and some eosinophilic cytoplasm. We performed immunohistochemical staining to rule out neuroectodermal tumor, neuroendocrine tumor, melanoma, lymphoma, and so on. However, the staining results were negative for pancytokeratin, CD3, CD20, CD99, chromogranin A, synaptophysin, CD56, ALK, HMB45, desmin, kappa, lambda, myoglobin, and S-100 protein. CT, MRI, and PET-CT were negative for extracutaneous involvement. Total excision was done, and additional immunohistochemical staining was performed to confirm the origin of the tumor. Staining results for vimentin, LCA, CD4, and CD30 were positive. We concluded that these findings were consistent with the small cell variant CD30+ PCALCL, which occurs rarely.

A Case of Primary Cutaneous CD30+ Anaplastic Large Cell Lymphoma, Small Cell Variant / 대한피부과학회지

Primary cutaneous anaplastic large cell lymphoma (PCALCL) is a rare cutaneous T-cell lymphoma. Most cases are composed of large anaplastic cells. However, it presents a wide spectrum of histologic patterns. In the small cell variant, a small-sized pleomorphic cell morphology can be seen. A 74-year-old woman presented with an 8-month history of asymptomatic ulcerative plaque and satellite nodule on the right calf. Her past medical history was not specific. The histologic findings on punch biopsy specimens showed a malignant small round cell tumor on both lesions. The tumor cells had large pleomorphic nuclei with multinucleation and some eosinophilic cytoplasm. We performed immunohistochemical staining to rule out neuroectodermal tumor, neuroendocrine tumor, melanoma, lymphoma, and so on. However, the staining results were negative for pancytokeratin, CD3, CD20, CD99, chromogranin A, synaptophysin, CD56, ALK, HMB45, desmin, kappa, lambda, myoglobin, and S-100 protein. CT, MRI, and PET-CT were negative for extracutaneous involvement. Total excision was done, and additional immunohistochemical staining was performed to confirm the origin of the tumor. Staining results for vimentin, LCA, CD4, and CD30 were positive. We concluded that these findings were consistent with the small cell variant CD30+ PCALCL, which occurs rarely.

Clinical treatment of primary cutaneous anaplastic large cell lym-phoma / 中国肿瘤临床

Objective: To explore the clinical presentation and therapy of primary cutaneous anaplastic large cell lymphoma (PCALCL). Methods:We reviewed and analyzed six cases of PCALCL who were treated at our hospital from January 2009 to Decem-ber 2012. Results:The subjects were three males and three females with a median age of 54 years old (range:38 years to 60 years). Three patients had a single subcutaneous nodule and three had multiple nodules. The subcutaneous lesions appeared red, solid, and sta-ble nodules. Some patients developed pruritus around the lesions, or developed more nodules, which coalesced with other nodules. Some of the lesions exhibited partial spontaneous regression and new nodules appeared at the same site or at different sites. Finally, the lesions changed color from red to brown. The surfaces of some lesions developed ulcerations. Only 1 case had superficial lymph node metastasis. Three cases with single lesions received surgical excision plus radiotherapy, chemotherapy, or radiochemotherapy;all three cases survived and achieved complete remission. The three cases with multiple lesions mainly received systemic chemotherapy, with two of the cases recurring and one surviving with complete remission. The median follow-up was 24 months (range: 11 months to 35 months), with a progression free survival of 66.7%and an overall survival of 100%. Conclusion:Surgical excision or radiotherapy provides satisfactory control in patients with single lesions. Chemotherapy may be prescribed to patients with multiple lesions or with extracutaneous metastasis.

Primary Cutaneous Anaplastic Large Cell Lymphoma of the Lower Lid

PURPOSE: Primary cutaneous anaplastic large cell lymphoma is rarely encountered in the lower eyelids. We report a patient with primary cutaneous anaplastic large cell lymphoma arising from the lower eyelid. METHODS: A 39-year-old man presented with a relatively fast growing mass on the center of his left lower eyelid for one month. The mass did not respond to local injection of triamcinolone at a local clinic. The lesion appeared as a solitary reddish nodule with ulceration, was non-tender, round, crusted, and measured 13 mm x 11 mm x 5 mm. Well- developed superficial vessels were found on the surface of the nodule. An incisional biopsy was performed. RESULTS: Histologic examination revealed that the bulk of the infiltrate was in the papillary and reticular dermis. Tumor cells had abundant, well-defined cytoplasm and pleomorphic nuclei with multiple nucleoli. The majority of the neoplastic cells showed immunoreactivity for CD 30 (Ki-1) along the cell membrane. A histopathological diagnosis of primary cutaneous anaplastic large cell lymphoma was made. CONCLUSIONS: Most cases of primary cutaneous anaplastic large cell lymphoma arise from the body and extremities. However, since primary cutaneous anaplastic large cell lymphoma may occur in the eyelid, it should be differentiated from nodular and relatively fast growing inflammatory tumors despite local steroid treatment.