[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).


CONCLUSIONS
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.
Lü
Zheng
Z
Department of Medical Oncology, Cancer Institute and Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100021, China.
Shi
Yuan-kai
YK
Zhou
Li-qiang
LQ
Qin
Yan
Y
Dong
Mei
M
Yang
Jian-liang
JL
Liu
Peng
P
Zhang
Chang-gong
CG
D000293:Adolescent; D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D019002:CD56 Antigen / Q000378:metabolism; D002648:Child; D003131:Combined Modality Therapy; D003520:Cyclophosphamide / Q000627:therapeutic use; D018572:Disease-Free Survival; D004317:Doxorubicin / Q000627:therapeutic use; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008206:Lymphatic Diseases / Q000209:etiology; D051359:Lymphohistiocytosis, Hemophagocytic / Q000209:etiology; D016399:Lymphoma, T-Cell / Q000150:complications / Q000188:drug therapy / Q000378:metabolism / Q000532:radiotherapy / Q000601:surgery; D008297:Male; D008875:Middle Aged; D015434:Panniculitis / Q000150:complications / Q000188:drug therapy / Q000378:metabolism / Q000532:radiotherapy / Q000601:surgery; D011241:Prednisone / Q000627:therapeutic use; D012189:Retrospective Studies; D015996:Survival Rate; D014750:Vincristine / Q000627:therapeutic use; D055815:Young Adult
D019002:CD56 Antigen; D014750:Vincristine; D004317:Doxorubicin; D003520:Cyclophosphamide; D011241:Prednisone