| PurposeThe present study investigates the clinicopathologic characteristics, imaging findings and immunophenotype of Dermatofibrosarcoma protuberans (DFSP), including the general characteristics in the recent five years as well as diagnosis and differential diagnosis. In advance, we are aiming to evaluate the myxoid variant of DFSP with emphasis on diagnosis and differential diagnosis.Methods1.514cases of DFSP were retrieved from the files of the Department of Pathology, Cancer Hospital, Fudan University between January2008and December2012. All the histological slides were reviewed for confirmation of pathologic diagnosis by two professional pathologists. The clinical features, imaging features, histological features and immunohistochemical features were analyzed with a independent preliminary study of myxoid variant.2. EnVision imunohistochemistry was performed to characterize the immunophenotype of DFSP, using a panel of antibodies including CD34, vimentin, S-100, EMA, AEI/AE3, desmin-SMA, MSA, caldesmon, calponin, P53, PGP9.5, PS2, bcl-2, ba x, MDR, TOPOⅡ α, GsTn, P27, cyclinDl, Ki-67, PCNA, ER, PR, etc.Results1.Clinical characters:(1)Gender:There were308male patients(59.92%) and206female patients(40.08%)(male to female;1.5:1).(2)Age:The age of the patients ranged from4to87years old with the median age of38years (average38.7±13.1years).74.3percent of patients were aged20-50years. There’s only one missing data.(3) Site:The site of three cases remained uncertain. The most common sites of tumor were the trunk, a total of359cases (70.3%,359/511), of which107cases occurred in the chest (20.9%,107/511),107cases occurred in the abdomen (20.9%,107/511),38cases on shoulder(7.4%,38/511),67cases on back(13.1%,67/511) and3’5cases on waist ((?).8%,37/511). The fllowing sites were head&neck and extremities. Both of them were66cases,accounting for12.9%(66/51(?))of all cases.20cases occurred in the uncommon site, including sixteen cases of the inguinal region, and six cases of the vulva.(4) Clinical findings:The vast majority of the cases presented with a local slow-growing tumor which were found unintentionally. Clinical manifestations were seldom noticed. The lesions showed normal epidermis, but red swelling of the skin presented in19cases, itching presented in7cases, pain or tenderness presented in6cases, erosion and ulceration presented in11cases, some of which might be related to scratch.3cases occurred after trauma and only1case occurred in pregnancy. The course of the patients ranged from0.2months to42years in all514cases.(5)Imaging findings:X-ray showed the soft tissue shadow, CT and MRI could clearly show the mass, the latter was usually considered as first choice. On T1WI the lesions exhibited intermediate to low signal intensity, whereas on T2WI, they were high to low signal intensity according to the quantity of the melan pigments. Isotopes and PET-CT scan helped to identify metastasis. Some case were alive with blood supply in MRI.(6)Treatment:All patients were treated with tumor resection and most of them didn’t have any aftertreatment;(7)Prognosis:The follow-up data showed that77cases had local recurrence with a time of1week to13years (average,64±33.29months), among which23cases showed repeated relapse.2cases had lung metastasis, none died of the tumor. Accelerated growth often occurred after the period of slow-growth in recurrent patients.2. Pathologic findings:(1)Gross findings:All except16were described as a solitary firm mass, with a nodular or lobulated appearance. Multiple lesions showed no obvious difference with the single one. The tumor measured from0.3to19.0cm in diameter (average,3.57cm)with grayish color on cut surfaces. Myxoid, fish-like, hemorrhage, necrosis or cystic changes were seen in some cases.(2)Histological findings:The classical tumors were composed of uniform spindle cells arranged in storiform or wheel-like that separated by thin fibrous septa. On light magnification, the tumor consisted with a few number of subtypes, including classical, fibrosarcomatous, myxoid, pigmented, atrophic and sclerosing change. More than two kinds of variants were seen in the same tumor simultaneously in a few cases. The mitotic figures ranged from0/10HPF to5/10HPF(mean,1/10HPF)3Immunohistochemical findings: CD34(149/153,97.4%),Vimentin (92/92,100%), Actin (12/133,9.0%), Bcl-2(49/91,53.8%), desmin (1/106,0.9%), KP1(2/52,3.8%), P53(12/34,35.3%), S-100(17/93,18.3%),etc.Conclusions1. Dermatof ibrosarcoma protuberans is a relatively rare soft tissue tumor mainly occurs in middle-aged adults with a slightly male predilection.2. The most common site was the trunk, and the chest and back were by far the most common single location of the tumor, followed by extremities, head and neck, and a small number of cases may occur in vulva and other positions. The vast majority of cases showed a locally slow-growing tumor.3. X-ray often showed the soft tissue shadow and CT and MRI examination could clearly show that mass. Isotopes and PET-CT could find metastasis.4. The tumors usually presented as subcutaeous nodules. Histologically, the tumor cells were spindle-like ethier short or long,with a storiform-like.Other uncommon histological changes like myxoid change, fibrosarcoma and pigmented area were noticed in about25%cases. The mitotic figure was scarce.5. Immunohistochemically, the tumor cells of DFSP were detected intense expression of CD34protein and Vimentin. None or few expression of biomarkers from myogenic, epithelium or nerve origin were noticed, such as α-SMA, desmin, AE1/AE3, NSE and NF.6. The overal prognosis is fine but local recurrence rate is high. |
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