The course and prognosis of pemphigus in 47 Tunisian patients.

BACKGROUND: Pemphigus is a severe and life-threatening autoimmune bullous dermatosis. OBJECTIVE: We have analysed parameters that may influence prognosis of pemphigus (P). METHODS: It was a retrospective study (2002-2010), with pemphigus considered as severe if body surface involvement ≥ 30%. Disease control and relapse-free survival (Kaplan-Meier) were analysed and compared according to several parameters (P < 0.05). RESULTS: 47 cases of pemphigus were collected, mean age 51 years ± 16.8 (F/H = 3.27). There were 30 pemphigus profundus and 17 superficial pemphigus. The median remission period was of 9 months (1.2 months-5 years). The mean healing time was of 40 days (6 days-4 months), which did not depend on type of P, its severity or infectious complications, whereas it was shorter in aged patient (≥ 65 years) compared to non aged ones (P = 0.018). 36.2% of patients had relapsed. Relapses were significantly more frequently observed only in the presence of mucosal involvement at presentation (P = 0.015). The median overall 1st relapse-free survival was of 2.33 years. Only mucosal involvement at presentation was associated with a shorter median 1st relapse-free survival time (1.28 years vs. 3 years) (P = 0.0017). Mortality rate was of 10.6% (n = 5); in four patients the death was directly related to pemphigus and occurred rapidly after the onset of lesions. CONCLUSION: Our study illustrates the poor prognosis of pemphigus by a long duration to disease control, a high initial dose of oral steroid, a high rate of relapse and a short remission period. Only mucosal involvement at presentation was identified as a poor prognostic factor.

[Ileocolic intussusception from metastatic renal cell carcinoma]. / Localisation iléale d'un carcinome à cellules rénales révélée par une invagination iléo-cæcale.

The lung, the liver, the bone tissue and the brain are the most frequent sites for renal cell carcinoma metastasis. Small bowel metastasis from renal cell carcinoma is rare, with only few cases published. We report the case of ileal metastasis from operated kidney cancer revealed by ileocolic intussusception and causing intestinal obstruction in a 32-year-old woman.

[Pediatric mandibular ameloblastic fibro-odontoma]. / Fibrome odonto-améloblastique de l'enfant.

INTRODUCTION: Ameloblastic fibro-odontoma (AFO) is a very rare mixed odontogenic tumor (2% of all odontogenic tumors). It is considered as a non-extensive non-aggressive tumor but recurrence and malignant transformation remain possible. We report a voluminous AFO in a child. CASE REPORT: A 7-year-old boy presented with a right mandibular and facial swelling. The panoramic radiograph and CT scan revealed a voluminous unilocular radiolucent lesion (10 cm) with an impacted tooth that involved the ramus area and the angle of the right mandible. Enucleation and curettage was performed with a good outcome. There was no recurrence at ten months. DISCUSSION: This case is exceptional because of the young patient's age, the large tumor diameter, and the good outcome after ten months. This tumor occurs more often in the posterior region of the mandible and is frequently seen in the first two decades of life. The diagnosis is made on radio-clinical data and may be confirmed by histology. A surgical treatment with complete tumor removal is recommended to prevent recurrence.

[Cecal perforation in a kidney transplant patient: disseminated histoplasmosis]. / Perforation caecale chez un transplanté rénal.

Disseminated histoplasmosis is a fungal infection caused by Histoplasma capsulatum. It often involves immunodeficient patients and can occur in two forms, i.e., the large- and small-celled variants. The purpose of this report is to describe a case of disseminated histoplasmosis with cutaneous and digestive involvement observed four years after kidney transplantation in a man from Senegal. The patient developed severe sepsis secondary to colonic perforation. Outcome was fatal due to delayed diagnosis and extent of disease.

[Tuberculous lupus]. / Le lupus tuberculeux.

UNLABELLED: Cutaneous tuberculosis accounts for 2% of all extrapulmonary tuberculosis and is one of the multiple clinical presentations of extrapulmonary tuberculosis. We report a case of cutaneous tuberculosis with unusual clinical and histological presentations. CASE REPORT: We report the case of a 29-year-old woman with plurifocal tuberculous lupus. Histological examination of cutaneous lesions revealed the presence of a granuloma with caseum necrosis. Chest X-ray showed a severe pulmonary tuberculosis lesion. The Mantoux test performed by intradermal injection was positive. Skin lesion PCR and sputum culture were negative. Both skin and pulmonary lesions responded to antituberculous treatment. COMMENTARY: Lupus vulgaris is commonly presents on the head and neck. The diagnosis may be difficult when lupus vulgaris occurs at unexpected sites or in unusual clinical presentations. Caseum necrosis observed in our patient was unusual.

[Gastric cryptosporidiosis revealing a small cell lung carcinoma (Tunisia)]. / Cryptosporidiose gastrique révélant un carcinome a petites cellules bronchique (Tunisie).

Cryptosporidium, agent of cryptosporidiosis, is an ubiquitous protozoan organism causing diarrhoea especially in severe immunosuppressed patients. Cryptosporidium has been detected with increasing frequency in the gastrointestinal tract, but involvement of the stomach is rarely reported and discloses an underlying immunodeficiency state. We report the case of 67-year-old man, a heavy smoker, who presented with a history of epigastric pain with an altered general condition. Upper gastrointestinal endoscopy showed no significant mucosal abnormalities. The biopsy revealed a chronic active gastritis with Cryptosporidium parasites lining cryptic epithelium. Systematic chest X ray showed a right suspect parenchymatous opacity. Bronchoscopy with multiple biopsies concluded to a small cell lung carcinoma. Through this rare initial manifestation of immunocompromised state related to cancer we will discuss the role of gastrointestinal endoscopy with biopsies in the diagnosis of cryptosporidiosis.

[Proliferating Brenner tumor: case report]. / Tumeur de Brenner proliférante: à propos d'un cas.

Brenner tumors account for only 1 to 2% of ovarian tumors. Proliferating Brenner tumors are characterized by an epithelial proliferation, resembling superficial well-differentiated urothelial carcinomas. A single 29-year-old patient was operated for an ovarian tumor. On gross examination, the tumor, which originated in the left ovary, was cystic multilocular and presented polyoid excrescences. Definitive histological exam showed a papillary proliferation lined by transitional cells without stroma infiltration. Mucinous cells lined some cavities. The diagnosis of proliferating Brenner tumor was made. Clinical and pathological characteristics of this rare entity will be discussed.

[Immunohistochemical study of endometrial stromal sarcoma and smooth-muscle tumors of the uterus]. / Etude immunohistochimique des sarcomes du stroma endométrial et des tumeurs musculaires lisses de l'utérus.

OBJECTIVES: Positive and differential diagnoses of mesenchymal tumors of the uterus may be sometimes problematic. The purpose of this study was to evaluate the utility of a panel of antibodies in this diagnosis. MATERIALS AND METHODS: The expression of AML, desmin, h-caldesmon and CD10 was studied in nine endometrial stromal sarcomas (SSE), two leiomyosarcomas (LMS) and 10 highly-cellular leiomyoma (HCL). RESULTS: AML positivity was found in five SSE, in all HCL and in only one LMS. Desmin expression was found in two SSE, in all HCL and LMS. H-caldesmon was negative in all SSE, positive in all HCL and in one case of LMS. CD10 was expressed in two-third of SSE. However, neither HCL nor LMS was marqued. CONCLUSION: CD10 is the most specific antibody of SSE. It seems to have the best value in the diagnosis of mesenchymal tumors in association with desmin and h-caldesmon, specific markers of smooth-muscle differentiation.

[Prognostic value of morphologic subdivision of papillary renal cell carcinoma and MUC1 expression]. / Intérêt pronostique du sous-typage morphologique des tumeurs tubulopapillaires du rein et expression de MUC1.

OBJECTIVE: The purpose of our study was to demonstrate the prognostic value of morphologic subdivision of papillary renal cell carcinoma and compare MUC1 expression in two types. MATERIALS AND METHODS: The present retrospective study included 30 cases of papillary renal cell carcinoma based on review of histology slides. The histologic type, Führman grade and stage pTNM were specified. Immunohistochemistry was performed in 22 cases using antibody MUC1. RESULTS: Patients were 23 men and seven women with a mean age of 59.6 years. Eleven tumors were type 1 and 19 were type 2. Type 2 tumors were significantly associated with a higher Führman grade (p = 0.0002). We showed a differential expression of MUC1, which is frequently expressed in the type 1. A local recurrence occurred in one case, lung metastasis in the second one and both tumors were type 2. The survival rate without recurrence and metastasis was 100 % in the type 1. It was 79 % at 12 months and 59 % at 24 months in the type 2. MUC1 expression was correlated with the outcome. CONCLUSION: Type 2 tumors are associated with a higher Führman grade than type 1 and MUC1 expression is more frequent in type 1 and correlated with outcome.

Granuloma annulare.

AIM: Granuloma annulare (GA) is a palisading granulomatous skin disease. It is a relatively common dermatosis typically characterized by an annular arrangement of erythematous or flesh-coloured papules. The aim of this study was to assess the epidemiological and clinical pattern of GA and its management. METHODS: A retrospective study dealing with 35 cases of GA collected during an 11-year period from 1995 to 2005 was carried out at the Dermatology Department of Charles Nicolle's Hospital of Tunis. RESULTS: There were 24 females and 11 males including 13 children. Four clinical patterns were observed: localized GA with annular shaped lesions in 22 cases (62.8%), subcutaneous GA in 9 cases (25.7%) with 4 children, combination of localized and subcutaneous patterns in one child (2.8%), generalized GA in one adult-patient (2.8%) and combination of generalized and subcutaneous patterns in 2 cases (5.7%). Associated diabetes mellitus was seen in 9 adults (25%) and thyroid diseases in 2 other adults (5.7%). Topical steroids were proposed in 9 cases with partial resolution in 6 of them. Four patients were treated by hydroxychloroquine with an improvement in 3 of them. Two patients (generalized GA with subcutaneous form and subcutaneous GA) were treated by dapsone with partial clearing. CONCLUSION: In these series, GA has the same epidemiological and clinical pattern as other series of the literature. Subcutaneous form had a pseudo-rheumatoid presentation in adults and an exclusive involvement of children when it is localized on the scalp. Associated systemic diseases, especially diabetes mellitus, are frequent in the series here observed.

Low-dose X-ray depilatory treatment induces trichoblastic tumors of the scalp.

BACKGROUND: Depilatory radiotherapy for ringworm was largely used before antifungals were available. Patients who underwent this treatment are at high risk of developing scalp tumors or other cancers. The aim of this study was to characterize scalp tumors occurring after X-ray therapy for ringworm. METHODS: We included cases of postradiotherapy scalp tumors recorded at the Dermatology Department of the Charles Nicolle Hospital, Tunis between 1988 and 2001. We recorded clinical descriptions and all cases were resubmitted to microscopic analysis. RESULTS: Sixty-one tumors occurred in 33 men and 12 women with a mean age of 49.8 years. Radiodermatitis was present in 21% of patients. Tumors were basal cell carcinomas in 47 cases, trichoblastomas in 10 cases and trichoblastic carcinomas in 4 cases. Twelve patients had 2-5 tumors, with combinations of tumor types in 3 of them. Mean delay of onset of tumors after radiotherapy was 39.4 years in basal cell carcinoma cases, 38.3 years in trichoblastoma cases and 35.6 years in trichoblastic carcinoma cases. CONCLUSIONS: This series shows that although basal cell carcinoma is the most frequent tumor in this situation, trichoblastomas are common. We describe, for the first time, radio-induced trichoblastic carcinomas. Trichoblastic tumors have not yet been described in this context because this concept is relatively recent.

[Paratesticular pleomorphic rhabdomyosarcoma in an adult: diagnosis and management]. / Rhabdomyosarcome pléomorphe paratesticulaire de l'adulte: diagnostic et prise en charge.

A paratesticular pleomorphic rhabdomyosarcoma occurring in a 63-year old man is reported. Clinical signs were like any intrascrotal tumor. CT-scan and histological examination with immunohistochemical study were necessary for the diagnosis and stadification of this cancer. The patient underwent a radical inguinal orchidectomy. The patient developed a local recurrence, which was treated by radiotherapy, and subsequently developed metastases two years later, which were treated by chemotherapy.

[Contribution of cell cycle and DNA content study by flow cytometry in the prognosis of superficial bladder cancer: Tunisian experience]. / Apport de l'étude du cycle cellulaire et du contenu en ADN par cytométrie en flux dans le pronostic des tumeurs superficielles de la vessie : expérience tunisienne.

Clinico-pathological study of superficial bladder cancer (pTa/pT1) informs about prognostic factors such as the size of the tumor, its uni or multifocal character, its grade and stage. Presently, these factors constitute the basis of therapeutic decision but do not allow to foresee the prognosis with certainty. Many technics have contributed to a better knowledge of such tumors; however, they have not allowed to fully master prognostic uncertainties. During the last decades, cell cycle and DNA content study by flow cytometry has been developping, bringing an additional prognostic element to various types of tumors. We have decided to study the impact of this technique to the assessment of the prognosis of superficial bladder tumors. The study concerned 65 patients presenting superficial bladder tumors (pTa/ pT1), with a follow-up of at least two years in case of not recurrence and in case of recurrence, having had a second resection with analysis of sections. Flow cytometry was applied to formol-fixed and paraffin-embedded endoscopic resection material of initial tumors by simple-labelling of DNA with propidium iodide. Following cytometric study, 35 (54%) of tumors were aneuploid and 30 (46 %) were diploid. For the diploid ones, S-phase mean value was 14.94% (from 2.72% to 33.43%); and G2M mean value was 8.3 (from 1% to 18%). The presence of an DNA- aneuploid peak had a predictive value of recurrence and progression in stage, with relative risks of 12 and 6.85 respectively. It was also correlated with the histological grade and stage. On the other hand, S-phase and G2M values had no prognostic significance.

[Beta 2-microglobulin amyloidosis presenting as intestinal perforation in a haemodialysis patient]. / Amyloïdose de type beta2-microglobuline révélée par une perforation intestinale chez une hémodialysée chronique.

Long term haemodialysis patient is subject to several complications such as generalised amyloidosis which is the result of deposits of beta2-microglobulin not depurated by haemodialysis. Digestive location causes ischemic accidents such as ulcer, infarctus, digestive haemorrhage, pseudo-obstruction and perforation manifested by a surgical emergency. Our observation is the 6th case of intestinal perforation caused by amyloidosis deposit reported in the literature.

[Adenoid cystic carcinoma of the trachea]. / Carcinome adénoïde kystique de la trachée.

UNLABELLED: Cystic adenoid carcinoma of the trachea, also called cylindroma, is a rare malignant tumor accounting for 1% of all respiratory tract cancers. OBJECTIVES: To recall through two observations and a review of the literature, various epidemiologic anatomical clinical aspects, and evolutionary of cystic glandular carcinome of the respiratory tracts. We report 2 cases of cystic glandular carcinoma of the trachea. CLINICAL CASE: They were two women of 60 and 49 year old who came with a deterioration of the general status with dyspnea, dysphonia and/or dysphagia. Radiological explorations visualized, in the first case, a tumoral process of the superior half of the trachea and, in the second case, a tumoral circumferential process budding in the sub-glottic area and reaching the first rings of the trachea. The two patients had a removal of the tumour An auxiliary radiotherapy was indicated in the first case. CONCLUSION: Cystic adenoid carcinomas of the trachea are rare tumours. Their diagnosis is based on the bronchial fibroscopy associated with the biopsy. The optimal treatment is surgical associated with the radiotherapy. The palliative treatments keep a place among inoperable patients.

[Study of the ploidy and cellular cycle of hidradenomas and hidradenocarcinomas: a series of 13 cases]. / Etude de la ploïdie et du cycle cellulaire des hidradenomes et des hidradenocarcinomes: a propos d'une série de 13 cas.

AIMS: To study by flow cytometry (FCM) the ploidy and the cellular cycle of nodular hidradenoma (NH) and hidradenocarcinoma (HC) and to assess the prognostic utility of this technique in such tumors. METHODS: We studied retrospectively 2 HC and 11 NH one of which was considered as an atypical NH. Monoparametric study by FCM was realized on paraffin-embedded material. The extracted cells were marked by Propidium's lodure and cellular cycle was analyzed by the software Mod-Fit LT. RESULTS: Our study showed eleven 100% diploid profiles, 10 of which had low S-phase varying between 2 and 12%. All of these 11 tumors were NH. S-phase was high (23.79%) in a single case that corresponded to the atypical NH. Two tumors showed aneuploid profiles; these corresponded to the 2 HC. CONCLUSION: The results of the cytometric study suit perfectly to those of the histopathologic examination. FCM could so help to establish the prognosis of these tumors. But further studies are necessary to determine the value of this technique.

Angiomyofibroblastoma of the spermatic cord: a case report.

Angiomyofibroblastoma (AMF) is a rare benign mesenchymal tumor with tendency to arise in the lower genital tract of middleaged women, predominately in the vulva. A few cases of AMF in males have been reported involving the scrotum, perineum or spermatic cord. We report a new case of AMF arising in the right inguinal region of a 27-year-old man. The tumor was well-circumscribed, myxoid and measured 30 mm in maximum dimension. On microscopic examination, the tumor was composed of spindle cells without atypia and with less than one mitosis figure per 10 high-power fields. Multinucleated cells and mast cells were observed. The stroma was myxoid and edematous with abundant capillary-sized blood vessels. Immunohistochemical staining showed a strong immunoreactivity for desmin and smooth muscle actin. The tumor cells were negative for estrogen receptors and focally positive for progesterone receptors with a low proliferative index of Ki67 (< 5%). This unusual neoplasm should be distinguished from aggressive angiomyxoma and other myxoid malignant tumors.

[Endometrioid adenocarcinoma of the uterine cervix associated with mucinous ovarian cystadenocarcinoma]. / Association d'un adénocarcinome du col de type endométrioïde à un cystadénocarcinome mucineux de l'ovaire.

The authors report the case of a 40-year-old woman, who was operated for an ovarian mucinous cystadenocarcinoma. The pathologic findings of the hysterectomy specimen with bilateral salpingoophorectomy showed an ovarian mucinous cystadenocarcinoma associated with an endometrioid adenocarcinoma of the uterine cervix. The mucinous cystadenocarcinoma represents the third most common type of ovarian carcinoma. In the literature, this tumor had been found in association with endocervical adenocarcinoma or with minimal deviation adenocarcinoma (adenoma malignum) of the uterine cervix. However, its association with an endometrioid adenocarcinoma, to our knowledge, has not been reported.