[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.

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.

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.

[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.

[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.

[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.

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.

[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.

[Extragastrointestinal stromal tumors: a report of 4 cases]. / Les tumeurs stromales extradigestives: à propos de quatre observations.

Gastrointestinal stromal tumors (GIST) are mesenchymal tumors that arise from the wall of the gastrointestinal tract expressing CD117 and/or the CD34. Similar tumors were described in the soft tissue of the abdomen and are so-called extragastrointestinal stromal tumors (EGIST). We report 4 new cases of EGIST. The tumors occurred in 2 women and 2 men, who ranged in age from 42 to 71 years. Three tumors arose from the soft tissue of the abdominal cavity, and the remainder arose from the retroperitoneum. They ranged in size from 10 to 27 cm. Three cases were composed purely of short fusiform cells tumors, the last case showed an epithelioid pattern. All the tumors expressed CD117. One patient presented with local recurrence and metastasis, one was lost to the follow-up and two patients were doing well. Immunohistochemistry is useful in distinguishing EGIST from other mesenchymal tumors. Intra-abdominal aggressive fibromatosis may express actin and CD117 but catenin is also positive, leiomyosarcoma expresses the actin and/or desmin but CD117 is usually negative, retroperitoneal dedifferentiated liposarcoma is CD117 negative and PS100 positive, inflammatory myofibroblastic tumor is negative for CD117 and CD34. The solitary fibrous tumor expresses CD34 and is negative to CD117. Some tumors expressing CD117 such desmoplastic round cell tumor should not be confused with EGIST. On the occasion of these four observations, we will discuss the clinical aspects and the main differential diagnoses of this tumor.

[Low-grade central chondrosarcoma: difficult diagnosis in an adolescent girl]. / Chondrosarcome central de bas grade: un diagnostic difficile: à propos d'un cas.

Central chondrosarcoma of the tibia is exceptional, particularly in young patients. Low-grade tumors raise difficult problems for histological distinction with enchondroma. We report a case of grade 1 chondrosarcoma located in the upper portion of the tibia in a 17-year-old girl. After radical surgery, outcome was favorable with no recurrence or metastasis at three years follow-up. The distinction between low-grade central chondrosarcoma and enchondroma is one of the most difficult challenges in bone pathology. Clinical, radiographic and pathological data must be considered together to reach certain diagnosis.

[Toxic nodular goitre associated with papillary thyroid carcinoma and primary hyperparathyroidism]. / Association d'un goitre multinodulaire toxique, d'un carcinome papillaire de la thyroïde, et d'une hyperparathyroïdie primaire.

The association of a toxic multinodular goitre with papillary thyroid carcinoma and primary hyperparathyroidism is very rare. Only one case is reported in the literature, we present the second one. It is a 51 year old woman, who initially presented with a toxic nodular goitre. The diagnosis of primary hyperparathyroidism was made following renal complications and the discovery of papillary thyroid carcinoma was incidental during the surgical treatment of parathyroid adenoma. Whilst the association of those three diseases is exceptional, the coexistence of any two of them is relatively frequent without any known common etiopathogenetis. It is recognised that hyperparathyroidism can be found in hyperthyroid patients, but the diagnosis of hyperparathyroidism in these cases is very difficult. The fortuitous discovery of papillary thyroid carcinoma during parathyroid surgery has already been reported but in most cases it is a microcarcinoma. In patients presenting with hyperthyroidism the risk of an associated carcinoma is generally felt to be negligeable. However, this associations is not rare. The association of primary hyperparathyroidism, hyperthyroidism and papillary carcinoma of the thyroid is rare. However, the authors suggest that the presence of any of the pathologies should trigger a search for the other two.

[Virilism during pregnancy]. / Virilisme au cours de la grossesse: à propos d'un cas.

We discuss the causes of virilism in pregnancy and the impact of hyperandrogeny on the female foetus. We report a case of virilism in a 28-year-old, gravida 1, para 1 patient with normal pregnancy and review the literature. After conception, the patient had been well until the 18th week of gestation, when she developed signs of virilization; her serum testosterone was markedly elevated. She delivered of a normal male infant at term. After delivery, signs of virilization regressed with normalization of testosterone level but a wide mass of the left ovary that persisted for 8 months. The histological study of the tumor showed luteinized thecoma. Luteomas and hyper-reactio luteinalis were the principal causes of virilism in pregnancy, thecomas are rare.

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.

[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.

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.