[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.

Functional defects of peripheral regulatory T lymphocytes in patients with progressive vitiligo.

Auto-reactive cytotoxic T lymphocytes play a key role in the progressive loss or destruction of melanocytes in vitiligo but the mechanism underlying the loss of self-tolerance is unknown. A deregulation of regulatory T-cell biology has recently been suggested. The analysis of the suppressive effects of peripheral T regulatory cells in vitiligo patients revealed a functional defect in seven of 15 cases. This defect was strongly correlated with disease activity. The evaluation of the percentage of peripheral regulatory T lymphocytes did not reveal any intrinsic quantitative defect. Yet, a decrease in the percentage of such cells was noted in patients with progressive forms, suggesting a recruitment of regulatory T cells from the peripheral blood to the site of injury. This was further corroborated by the significant increase of Forkhead box P3 expression in the vitiliginous skin of patients. Our data support the involvement of a functional defect of peripheral regulatory T cells in the pathogenesis of vitiligo and open new possibilities to advance therapeutic approaches.

[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.

[The myofibroblastic inflammatory tumours: A fortuitous association or an unknown postpartum complication]. / Les tumeurs myofibroblastiques inflammatoires : association fortuite ou complication peu connue du post-partum ?

The inflammatory myofibroblastic tumour has clinical, biological or histological features sometimes misleading with a septic condition. Presenting symptoms are variable and arising circumstances remain obscure. We report three cases occurring in a postpartum context. The first patient, a 28-year-old female, had left psoitis with a sepsis the first day postpartum in relation with an inflammatory myofibroblastic tumour of the meso-ovary. The second patient, a 40-year-old woman, had a hepatic inflammatory myofibroblastic tumour revealed by a ruptured sub-capsular haematoma of the liver in the forth day postpartum. The third patient, a 32-year-old woman, had a pulmonary inflammatory myofibroblastic tumour, diagnosed 5 months after a delivery and which recurred 10 years after surgical treatment. These cases illustrate the difficulty to diagnose inflammatory myofibroblastic tumour, particularly in postpartum.

[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.

Rectal leiomyosarcoma: report on two cases and a practical approach to differential diagnosis.

Rectal leiomyosarcoma is an uncommon malignancy. Herein, we describe the clinicopathological features and biological behaviour of these tumours, and provide a practical approach to differential diagnosis, particularly with gastrointestinal stromal tumours (GIST). We report two cases in elderly men. In the first, the lesion was 2 cm from the anal sphincter, while it was located in the rectal ampulla in the second case. Histologically, both tumours were characterized by pleiomorphic, large spindle cells, presenting numerous mitoses and marked nuclear atypia. Immunohistochemical analysis showed that tumour cells coexpressed both actin and desmin, whereas CD117 and S100 protein were negative. The final diagnosis was leiomyosarcoma. One of the patients died of pulmonary metastasis within six months. The second patient had bone metastasis, but was lost to follow up. This report underlines the potential diagnostic problems raised by rectal leiomyosarcoma and emphasizes the role of immunohistochemistry in achieving correct diagnosis, which has important clinical, therapeutic and prognostic consequences.

Primary amelanotic anorectal melanoma: an uncommon neoplasia with poor prognosis.

BACKGROUND: Anorectal melanoma is a rare and aggressive mucosal cancer. There is usually a delay in diagnosis because about 30% of these cancers are amelanotic and are often mistaken for benign conditions. Herein, we report a case of amelanotic anorectal malignant melanoma with an unusual metastatic deposit in the vulva and also review the literature. CASE REPORT: A 67-year-old woman presented with a history of prolapse of an anal tumour. Clinical examination showed a pedunculated and ulcerated amelanotic tumour associated with three other nodules, 1 cm in diameter, localized in the vulval mucosa. A left inguinal node was palpable. Histological examination and immunohistochemical staining of all tumours demonstrated malignant melanoma. Radiological diagnostic procedures revealed no evidence of metastases. DISCUSSION: Nine cases of amelanotic malignant melanoma have been reported in the literature. The age at diagnosis ranged from 45 to 77 years. Females appear to be far more frequently involved than males (F/M = 7/2). Anorectal melanoma is most common in the rectum, followed by the anal canal. Metastases occur early. Our case is the tenth case of amelanotic anorectal melanoma and probably corresponds to multiple synchronous primary melanomas of the anorectal region and the vulva, with the possibility that one of the lesions is a primary melanoma and the others are satellite lesions. Wide local excision where negative margins can be achieved is the preferred treatment.

Effect of liver hypothermic preservation: exploration and protection.

BACKGROUND/AIMS: The principal aim of conservation is to maintain the viability of grafts. This requires the addition of a cellular protector allowing better conservation of the graft. The aim of this work is to evaluate the effect of trimetazidine (TMZ) addition to Wistar rat livers conserved in Krebs-Henseleit solution, compared to the livers preserved only in Krebs-Henseleit solution (24 h at 4 degrees C). METHODS: 40 Wistar female rats divided into 5 groups were used: the first group consists of nonpreserved livers, the second consists of livers preserved only in the Krebs-Henseleit solution, and the other 3 groups consist of livers preserved in Krebs solution with different concentrations of TMZ added (16.5, 49.5 and 165 microg/ml). RESULTS: The obtained results show an improvement in the state of the liver in the presence of a high concentration of TMZ, which approaches normal physiological conditions. We note a clear diminution of transaminase activities, as well as an amelioration in metabolic capacities of the liver if the mitochondrial esterase pathway is supported in Wistar rats by a reduction of histological injuries. CONCLUSION: A TMZ concentration of 165 microg/ml clearly restored the metabolic capacities of the liver. Indeed, TMZ limited the appearance of necrotic areas and almost suppressed apoptotic cells.

Urothelial carcinoma with plasmocytoid component.

Plasmocytoid urothelial carcinoma is a rare subtype of tumour of the urinary bladder. Its clinical and histopathological features have not been well characterized. There are few reports of this type of tumour. We report a case of 65-year-old man who was operated in our department for bladder tumour. The pathological diagnosis was high-grade urothelial carcinoma with plasmocytoid component. The patient died shortly thereafter from liver and bone metastasis.

No evidence of the APC D1822V missense variant's pathogenicity in Tunisian patients with sporadic colorectal cancer.

OBJECTIVES: Sporadic colorectal cancer is influenced by numerous single nucleotide polymorphisms (SNPs), each with minor effects on the cancer risk. This study seeks to determine whether there is any association of the I1307K, E1317Q and D1822V variants within the Adenomatous polyposis coli gene (APC) and risk to develop colorectal cancer in Tunisian population. METHODS: Direct sequencing was used to investigate three SNPs in the APC in 48 Tunisian sporadic colorectal cancer cases and 63 controls. RESULTS: There was no statistically significant association between the I1307K, E1317Q and D1822V variants investigated and colorectal cancer risk. CONCLUSION: The lack of association may show that these variants selected for this study are not involved in the colorectal carcinogenic process. Otherwise, the eventual biological effect is so little to go undetected, unless increasing the sample size.

[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.

[Primary bilateral adrenal T-cell lymphoma. A case report rarer than B-cell lymphoma]. / Lymphome surrénalien primitif bilatéral de phénotype T. Un cas clinique beaucoup plus rare que le lymphome B.

Primary adrenal lymphoma is a rare condition. Only 70 cases were described in the literature. Adrenal lymphoma is often bilateral and in most of the cases of B-cell type. T-cell lymphoma is exceptional. The prognosis is bad and patient can die early because of acute adrenal insufficiency. We report a case of a 70-year-old man who was admitted for acute adrenal insufficiency due to primary bilateral adrenal T-cell lymphoma. He had corticotherapy and surgical exploration for intra-abdominal sepsis. He died because of multivisceral deficiency. Clinical features and imaging are not specific. (18)F-FDG PET Scan is an excellent mean to detect malignant tumor of adrenal gland. Percutaneous needle biopsy is useful to determine histology. The standard treatment is chemotherapy.

[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.

Segmental infarction of the testis: an exceptional complication of diabetes microangiopathy.

We report a case of segmental infarction of the testis in a 55-year-old man. Past medical history included 12 years of type II diabetes and hypertension. The patient presented with a 2-month history of testicular pain and was found clinically and sonographically to have a testicular tumour. The pathological examination of the partial orchiectomy specimen revealed segmental infarction of the testicle secondary to diabetes microangiopathy. We propose diabetes microangiopathy as a localization and aetiology of segmental testicular infarction. A possible testicular sparing procedure through an inguinal approach may be considered in cases of testicular masses for which the clinical and imaging findings are suggestive of focal testicular infarction.

Primitive neuroectodermal tumor of the kidney with vena caval tumor thrombus: diagnosis and management.

Primitive neuroectodermal tumors (PNET) of the kidney are rare and highly aggressive malignancies. We report a case of 28-year-old male with PNET of the kidney with inferior vena caval thrombus. Immunohistochemistry revealed strong positivity for CD99 and weak positivity for vimentin. Neuron-specific enolase (NSE), chromogranin and cytokeratin were negative. Patient underwent nephrectomy and six cycles of polychemotherapy the patient was in partial remission. He underwent two further cycles of high dose chemotherapy and died 9 months after diagnosis due to liver metastases. The diagnosis of renal PNET must be considered in young patients with renal neoplasm, particularly those with advanced disease at presentation. Achieving exact diagnosis has important clinical consequences.

[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.

Cystic nephroma in the adult: pathological aspects and therapeutic implications.

Cystic nephroma is a benign renal neoplasm. Since its initial description, there has been much debate regarding its origin. Preoperative diagnosis of Cystic nephroma is difficult to achieve. The differential diagnoses of Cystic nephroma are recently described mixed epithelial and stromal tumours of the kidney and cystic renal cell carcinoma. The Authors report three cases of Cystic nephroma and illustrate the clinical, radiological and histological features of this renal neoplasm.