Subcutaneous Panniculitis-Like T-Cell Lymphoma With Granulomas as the Predominant Feature.

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare primary cutaneous lymphoma preferentially localized in the subcutaneous adipose tissue and composed of cytotoxic T cells with an α/ß immunophenotype. The neoplastic T cells can be variably admixed with other inflammatory cells, including histiocytes, which can rarely form noncaseating granulomas. We present a case of SPTCL in which granulomas are the predominant feature, composing 75%-80% of the inflammatory infiltrate. The top differential diagnoses included infectious and autoimmune etiologies. However, special stains for microorganisms were negative, and immunohistochemical analysis of the atypical lymphocytes showed a CD3, CD8, TIA-1+, T-cell receptor (TCR) beta+, and CD4 infiltrate with a high Ki67 proliferation index of approximately 30%. TCR gene rearrangement studies by polymerase chain reaction with confirmation by high-throughput sequencing were necessary to exclude an autoimmune etiology, specifically lupus erythematosus panniculitis. To the best of our knowledge, only 1 other case of SPTCL with prominent granulomas has been reported in the literature. It is important for dermatopathologists to recognize this presentation of SPTCL. SPTCL with predominant granulomas should be included in the differential diagnosis of granulomatous panniculitis along with infectious and autoimmune panniculitides as well as other granulomatous lymphomas.

[Primary subcutaneous panniculitis-like T-cell cutaneous lymphoma: clinical presentation, treatment and prognosis].

OBJECTIVE: To explore the clinical presentation, treatment and prognosis study of primary subcutaneous panniculitis-like T-cell cutaneous lymphoma (SCPTCL). METHODS: Ten cases of SCPTCL, treated in our hospital from January 1999 to January 2009, were included in this study. Their clinicopathological data were reviewed and analyzed retrospectively. RESULTS: the median age was 50.5 years (range: 10 - 58), 4 males and 6 females. There were seven CD56 positive, two negative cases and 1 unclear case. Four cases had repeatedly nodules regressed spontaneously without treatment before diagnosis and new nodules appeared at different sites. Seven patients presented with multiple subcutaneous nodules or deeply seated plaques, most commonly on the extremities and trunk. Ulceration of nodules occurred in 3 cases, and the lesions were painful in five cases. The lesions appeared nodules at the beginning, and then gradually grew into tumors. Four patients had abnormal liver function and one patient had hemophagocytic syndrome (HPS), four patients had lymphadenopathy or visceral involvement. Three cases with single lesion underwent surgical excision in combination with chemotherapy or chemotherapy/radiotherapy. One case lost follow up, and two cases live without disease. Among the seven patients with multiple lesions, lymphadenopathy or visceral involvement, one underwent local surgical excision and is alive without disease, six of them received chemotherapy or multi-modality treatment mainly with chemotherapy. Three of these 6 cases are alive without progression, one used histone deacetylase inhibitors after progression and obtained partial regression, and 2 died. The median follow-up for all the 10 patients was 44 months (range: 14 - 99). The progression free survival was 66.7% (6/9), and overall survival was 77.8% (7/9). CONCLUSION: SPTCL has an indolent course, some lesions can regress spontaneously and relapse again. Patients with single lesion may live long-term without disease after multimodality therapy. Patients with multiple lesions or extracutaneous involvement are sensitive to CHOP-like regimen, but the duration of remission is short. Histone deacetylase inhibitors may be a promising drug in the treatment for SCPTCL relapse.

[Clinical analysis of 19 cases of subcutaneous panniculitis T-cell lymphoma with literature review].

BACKGROUND AND OBJECTIVE: Subcutaneous panniculitis T-cell lymphoma (SPTCL) is a rare subtype of primary cutaneous lymphoma. This study was to analyze the clinical characteristics, treatment and prognosis of SPTCL. METHODS: Clinical data of 19 SPTCL patients, treated at Cancer Center and the First Affiliated Hospital of Sun Yat-sen University from January 2001 to July 2007, were analyzed. RESULTS: The median age of the patients was 36 years. Seven patients had skin-excluded extra-nodal involvement; ten had lactate dehydragenase (LDH) elevation before treatment; one had hemophagocytic syndrome. Most of the parents received chemotherapy, including CHOP regimen, modified-alternative triple therapy (m-ATT), and Hyper-CVAD/HD-MA regimen. The median follow-up was 56 months. The median survival was 40 months, and the 2-year expected overall survival rate was 56%. Eight patients who received treatment of intensive chemotherapy had continous remission of 17-70 months; six of them underwent radiotherapy after chemotherapy. Univariate analysis (log-rank test) showed that sex, B symptoms, skin-excluded extra-nodal involvement, and pre-treatment blood cell count and LDH level affected the prognosis. CONCLUSIONS: SPTCL might be cured by high dose chemotherapy combined with whole body irradiation. The regimens which are effective without crossing resistance or more intensive may improve the response rate and overall survival.