Dermatofibrosarcoma protuberans: clinicopathologic presentation in Nigerians

Abstract

IntroductionDermatofibrosarcoma protuberance (DFSP) is in general a rare low grade malignant sarcoma and possesses a tendency for local recurrence. It has a site predilection for the trunk. Occurrence in the facial area is extremely rare. Ample knowledge of its clinical, histological and biologic characteristics is vital for accurate and prompt recognition.MethodsOver 13 years, clinicohistologic information of cases was retrieved. Histological and immunohistochemical re-evaluation were performed to re-confirm diagnosis. Data collected and analyzed with SPSS Statistics version 20 were presented as frequency tables, charts and proportions as appropriate.ResultsOf 191 soft tissue sarcomas, a total of 28 cases were diagnosed as DFSP (14.7%). Facial types occurred in 3 cases (1.6%). Tumour had age and site predilections for the 4th decade and trunk respectively. There was an equal gender distribution among cases. Most common clinical presentation was in form of painless protruding nodular mass. General histologic presentation revealed cellular lesions composed of spindle to oval neoplastic cells arranged in a storiform pattern. Mitotic figures were rare. All cases showed positive expressions to CD34.ConclusionFacial DFSP is rare among Nigerians. Its clinical appearance may mimic other common benign lesions of the head and neck region often resulting in misdiagnoses. A comprehensive knowledge of its clinical and histologic presentations and biologic behavior, combined with its identification with the aid of advanced histologic and radiographic techniques results in prompt confirmatory diagnosis. Appropriate treatment should include adequate surgical excision techniques combined with adjuvant radiotherapy or chemotherapy.

Dermatofibrosarcoma protuberance in a black African cohort – a clinicopathologic study

“…This present study has shown a recurrence following 20 years after primary tumour resection and this may be one of the longest duration so far reported in literature with a few reporting 10 years [ 8 ], while cases showing recurrence few weeks after resection has also been documented [ 8 ]. Patients could therefore be expected to develop recurrence beyond the recommended follow up interval [ 6 ].We believe that this study is one of the few to report male predominance [ 5 ] compared to most studies reporting more cases in females [ 3 , 4 , 8 , 10 ], with a study in Nigeria showing equal gender incidence [ 7 ]. Another study that reported male predominance considered only acral site tumours, hence, the study may not be representative of all cases seen in that population.…”

“…The rarity of DFSP is shown by the small number of cases reported in various studies from different parts of the world, some of which span more than two decades [ 6 , 7 , 10 ]. This current study is another report in that regard.…”

“…Although DFSP is said to be seen more in middle-aged adults,[ 11 ] recent literature suggests a higher frequency among the younger population [ 3 ] as also shown in the present study, however that observation might be due to the relatively young population structure in Nigeria. The occurrence in individuals 5-year old or younger has been reported [ 7 , 10 ] with one being in south-west Nigeria [ 7 ]. Our study supports these findings with the majority being younger than 40 years, it also supports the finding by Kreicher et al [ 3 ] in the United States which reported modal age of occurrence at 20 to 39 years.…”

“…Studies have shown a higher occurrence among the white population in multiracial studies [ 3 , 4 ]. Gender predilection is variable [ 3 – 5 ] with some studies reporting equal sex occurrence [ 6 , 7 ].…”

“…It is, therefore, regarded as a low-grade sarcoma with excellent 10 year survival of 99% [ 2 , 6 ]. Studies on dermatofibrosarcoma protuberance from Nigeria are few [ 7 ], hence the need to undertake this study. More so, cases of missed diagnosis are frequent with patients often presenting as a recurrence before accurate diagnosis is made [ 8 , 10 ].…”