Dermatofibrosarcoma protuberans. About 18 cases

Dermatofibrosarcoma protuberans (DFSP) is a rare mesenchyma skin tumor. It is characterized by a slow growth with a high rate of recurrence but limited potential for metastasis. We report the result of a retrospective study of 18 cases of DFSP collected over 16 years in the dermatology department of La Rabta Hospital. The mean age was 32 years with a sex ratio of 1.57. The site of predilection was the trunk (77.5%). Histological features were typical in all cases with positivity for CD34 in 14 cases. Treatment was surgical in 13 cases. DFSP is a tumor of intermediate malignancy, which can have aggressive course. This tumor is best treated with surgery in which large excision are necessary to reduce the risk of recurrence.     

Gastrointestinal stromal tumor: epidemiology and outcome study in 40 cases

The aim of this study was to analyze epidemiology and outcome of 40 patients with gastrointestinal stromal tumor (GIST) expressed CD 117. All epidemiological data (age, sex, location) and survival data were analyzed retrospectively. The tumors occurred in 17 men and 23 women. Stomach was the most common site of localization (47.5%). Of the 37 patients without Gleevec treatment, 18 benignant cases (66.6%) showed long-term survival (2 to 8 years) and 9 malignant cases (33.3%) were diagnosed. 10 cases were still being in followed (< 2 years). Gastric tumor's survival was better than extragastric tumor's survival without statistically significance (p>0.05). Our experience confirms that GIST is an aggressive tumor. 33.33% of them are malignant with recurrence or metastatic potential. Additionnally, epidemiologic factors (age, sex and location) do not have a predictive value.     

Three cases of cutaneous blastomycosis

Blastomycosis is a rare mycotic infection in Africa. Isolated cutaneous disease is extremely rare. We report three cases of cutaneous blastomycosis confirmed by istologic and mycologic examination. No visceral involvement was found. Blastomycosis is rare in Tunisia. The most common site of involvement is the lung. The three cases reported presented with skin involvement only, suggesting a cutaneous inoculation of Blastomyces dermatitidis. The diagnostic criteria of cutaneous blastomycosis are discussed.     

Unusual presentation of visceral leishmaniasis in an immunocompetent patient

Visceral leishmaniasis due to Leishmania infantum is endemic in Tunisia. The incidence in adult patients has increased in recent years; but most of these patients are not HIV positive as in other Mediterranean countries where all the cases are associated with HIV. We present the case of a woman with symptoms suggestive of leishmaniasis but whose bone marrow was sterile and whose serological tests for Leishmania spp. were negative. For this patient, the parasite was only detected in a routine duodenal biopsy. There are few reports of visceral leishmaniasis cases diagnosed by duodenal biopsy and almost all patients were HIV positive in that case. Thus it seems interesting to perform a duodenal biopsy in case of a difficult diagnostic even in the absence of gastrointestinal symptoms and/or HIV infection.     

Intraductal papillary mucinous tumor of the pancreas degenerated and fistulisated in the stomach, the duodenum and colon

We report a case of intraductal papillary mucinous tumor of the pancreas (IPMT) degenerated and fistulized into the stomach, the duodenum and the colon. Diagnoses of malignancy and fistulization were proven preoperatively. Pancreaticoduodenectomy extended to the body was performed. Pathological examination revealed a malignant IPMT with an invasive component. Of the 3 fistulizations tracts, both gastric and duodenal ones were malignant. To our knowledge, this is the first reported case of IPMT with colonic fistulization. Twelve months later, the patient is alive without signs of recurrence.     

Endometrial adenofibroma]

We report a case of papillary adenofibroma of the uterine corpus in a 31 year-old woman who was initially examined for vaginal bleeding. Pelvic examination showed a large polypoid mass protruding through the cervix canal. A pelvic ultrasound revealed a polypoid cystic mass apparently arising from the uterus. A polypectomy was performed. The tumor was composed histologically by begin epithelial and mesenchymal components. Uterine adenofibroma is a extremely rare tumor which considered to be a mixed tumor of Müllerian origin. This lesion appears to be clinically and histologically benign but must be differentiated from other malignant lesions of the uterus, particularly from the adenosarcoma.