Faten Limaiem

Primary hydatidosis of the central nervous system: A retrospective study of 39 Tunisian cases

OBJECTIVE To analyze epidemiological characteristics, clinical symptoms, radiological aspects, treatment and outcome of central nervous system hydatidosis and compare our results with those reported in literature. PATIENTS AND METHODS In our retrospective study, we reviewed 39 cases of primary central nervous system hydatid cysts operated on in our hospital between 1998 and 2007. RESULTS There were 20 male and 19 female patients (sex-ratio M/F=1.05) between 2 and 68 years of age (mean=26.5 years). Thirteen of the patients were children (33.3%) with a mean age of 6.8 years and 26 were adults (66.7%) with a mean age of 36.3 years. The location of hydatid cysts was intracranial in 27 cases (69.2%) and spinal in 12 cases (30.8%). Headache and motor deficits were the predominant symptoms in patients with intracranial hydatidosis whereas back pain and spinal cord compression syndrome were the most frequent clinical presentations in patients with spinal hydatidosis. All patients underwent surgical resection of the cyst. Pathologic findings were consistent with hydatid cyst in all cases. During the follow-up period which ranged between 12 months and 5 years, 12 patients had recurrence (30.7%). Only one patient with intracranial hydatid cyst died postoperatively due to anaphylactic shock. CONCLUSION Despite all the advances in imaging techniques and therapeutic methods, central nervous system hydatidosis remains difficult to cure and patient outcomes are not satisfactory especially in case of spinal involvement due to the high incidence of recurrence.

Recurrent cerebellar liponeurocytoma with supratentorial extension.

Cerebellar liponeurocytoma (LPN) is a rare central nervous system tumour recently recognized as a distinct clinicopathological entity. It was included in the previous 2000 edition of the World Health Organization (WHO) classification as a grade I neoplasm under the heading of glioneuronal tumours1. With increasing recognition of this entity and longer clinical follow-up periods in its investigation, it has become clear that this tumour has a rate of recurrence that is higher than previously thought and not compatible with a grade I designation. In light of the substantial rate of tumour recurrences, the current 2007 WHO classification assigns the cerebellar liponeurocytoma to WHO grade II tumour2. In this paper, we report a new case of a recurrent cerebellar LPN with supratentorial extension despite persistently benign histological features. To the best of our knowledge, recurrent cerebellar LPN with supratentorial extension has never been reported before, as the nine cases of recurrent cerebellar LPN published to date were confined to the posterior fossa.

Primary orbital hydatid cyst in an elderly patient.

BACKGROUND Orbital hydatidosis is rare, accounting for only 1% of all hydatid cysts. Herein we report a case and review the sparse literature. METHODS Case report and review of pertinent literature. RESULTS A 74-year-old farmer presented with progressive proptosis and loss of vision of the left eye. Evaluation revealed a hydatid cyst of the orbit. Orbital hydatidosis is usually unilateral and unaccompanied by cysts elsewhere in the body. Most affected are children and young adults. Although there is no gender predominance, orbital hydatidosis may affect the left eye preferentially. Onset is usually insidious over several months. Serologic testing is unreliable, and imaging by computed tomography or magnetic resonance imaging is now standard. Surgical resection is curative, but albendazole must be administered postoperatively if there is spillage intraoperatively. CONCLUSIONS Hydatid cysts should be included in the differential diagnosis of unilateral proptosis in patients from regions where echinococcosis is endemic.

Pancreatic cystic neoplasms.

Background: Cystic neoplasms of the pancreas are rare and constitute approximately 0.5% of all pancreatic neoplasms. Aims: The study was to describe clinicopathological features of pancreatic cystic tumors. Patients and Methods: In our retrospective study, we reviewed 10 cases of pancreatic cystic neoplasms that were diagnosed at the pathology department of Mongi Slim hospital over a 14-year period (2000-2013). We adopted the latest World Health Organization (WHO) classification (2010) in grouping all tumors. Results: There were one male and nine female patients (sex ratio M/F = 1:9) aged between 21 and 68 years (mean = 37.5 years). The most common clinical presentation was epigastric and abdominal pain (n = 6) followed by vomiting (n = 3). Abdominal computed tomography (CT) scan disclosed a cystic lesion of the pancreas ranging in size between 2 and 10 cm (mean = 6.75 cm). All patients underwent surgical treatment. Histopathological examination of the surgical specimen established the diagnosis of solid pseudopapillary neoplasm (n = 2), serous cystic neoplasm (n = 2), mucinous cystadenoma (n = 4), mucinous cystadenocarcinoma (n = 1), and intraductal papillary mucinous neoplasm with invasive carcinoma (n = 1). Conclusion: Better understanding of pancreatic cystic neoplasms is essential for clinicians to make accurate diagnosis and to provide the best management for patients.

Hydatidosis of the Brain

Cerebral hydatidosis is a rare parasitic disease representing only 1–2 % of all cerebral space-occupying lesions. It is still a major health problem in infected areas of the world, especially in rural areas. The vast majority of patients affected are children. They present with a wide range of clinical manifestations which mainly depend on the location and size of a given hydatid cyst. Computed tomography and magnetic resonance imaging have greatly contributed to a more accurate diagnosis of hydatidosis. Yet the gold standard of diagnosis remains a pathological one. Extirpation of the intact cyst is the treatment of choice, resulting in most cases to a complete recovery. Despite the introduction of modern surgical and pharmacological therapy, brain hydatid disease continues to be a substantial cause of morbidity and mortality in many parts of the world. Such a serious health problem should enhance endemic areas to develop more efficient preventive measures throughout rural areas in order to reduce mortality rates due to this disease.

Primary orbital hemangiopericytoma: an unusual cause of unilateral proptosis.

numerous orbital neoplastic and non neoplastic processes. Various mesenchymal tumours of both fibrohistiocytic and vascular origin are well-described causes. Hemangiopericytomas (HPC) are rare vascular tumours which can infrequently involve the orbit and their incidence is estimated to be 0.8% to 3% of primary orbital tumours1,2. We herein report a new case of orbital HPC revealed by unilateral proptosis in a 38-year-old man. Our aim was to highlight the clinicopathological and radiological features of this rare neoplasm with review of the current literature.

Valeur de l’examen extemporané en pathologie thyroïdienne

AIM OF STUDY To assess the value of intraoperative frozen-section diagnosis in thyroid surgery and determine its limitations. METHODS This retrospective study examined the results of 1534 frozen sections of thyroid specimens analyzed over the 11-year period from 1995 through 2005 and their correlations with the final histological examination. Deferred responses were not taken into account for statistical calculations. RESULTS In our series, frozen-section diagnosis was concordant with subsequent histopathological examination in 92% of cases, discordant in 3%, and deferred in 5%. The global specificity of frozen section analysis for all histological subtypes was 99.8% and its sensitivity 67%. Its sensitivity for thyroid cancer was lower in follicular (42%) and papillary carcinoma (64%) than medullary (100%), anaplastic (100%), secondary (100%), mixed (100%), and insular carcinomas (86%). CONCLUSION In our series, as in the literature, most of the discordances between frozen-section and definitive diagnosis were associated with microfollicular lesions, which explain the low sensitivity of 67%. Our results demonstrate the reliability of intraoperative frozen-section analysis in thyroid surgery. Collaboration between pathologists, radiologists, and clinicians should be emphasized.

Carcinome lobulaire à cellules pléomorphes du sein. Difficultés diagnostiques: à propos d’un cas avec revue de la littérature

We report a case of a 27-years-old woman with a lesion at the right breast, incidentally noted. The patient underwent tumour resection followed by a right mastectomy type “Patey”. Microscopic analysis of the tumour had recognised an invasive pleomorphic lobular carcinoma. Invasive pleomorphic lobular carcinoma (PLC) is a distinctive aggressive subtype of invasive lobular carcinomas (ILC). It has the typical infiltrating pattern of classical ILC of diffuse single cell spread but the nuclei aremore pleomorphic. Histological differential diagnosis with ductal carcinoma may be difficult, but it is important for this difference to be done. E-cadherine is a trans-membrane glycoprotein, typically expressed in ductal carcinoma, and loss of E-cadherine expression characterises invasive and in situ lobular carcinoma.RésuméIl s’agit d’une patiente âgée de 27 ans, admise pour un nodule indolore du quadrant supéroexterne dusein droit découvert fortuitement. L’examen clinique du nodule n’objectivait ni de rétraction du mamelon, ni d’écoulement mamelonnaire, ni d’adénopathie axillaire. La patiente bénéficiait d’une tumorectomie suivie d’une mastectomie de type Patey. L’examen anatomopathologique concluait à un carcinome lobulaire à cellules pléomorphes. Le carcinome lobulaire du sein à cellules pléomorphes est une variétérare du cancer mammaire récemment individualisée, dotée d’un potentiel évolutif particulièrementagressif. Il s’organise sur le plan architectural comme un cancer lobulaire invasif, avec cependant, un pléomorphisme cytonucléaire trè s marqué posant des problèmes de diagnostic différentiel avec le carcinome canalaire infiltrant peu différencié. L’E-cadhérine est d’un apport considérable, elle permet en effet de trancher entre ces deux entités dont les modalités thérapeutiques et évolutives sont très différentes.

Une tumeur maligne primitive rarissime de la vésicule biliaire. Le carcinome épidermoïde

Carcinomas of the gallbladder are rare malignancies. They are mostly represented by adenocarcinoma. Pure squamous cell carcinoma is a rare histologic subtype characterized by a rapid growth, with local extension and a poor prognosis. We present a recent case of a 63-years-old man admitted with the complaints of pain in right hypochondrium. Computed tomography revealed a tumour in the gallbladder fossa region with infiltration of the liver. Cholecystectomy was achieved. Pathologic examination of the surgical specimen revealed a well differentiated squamous cell carcinoma.RésuméLes carcinomes primitifs de la vésicule biliaire sont des tumeurs peu fréquentes. Ils sont essentiellement représentés par l’adénocarcinome. Le carcinome épidermoïde représente un type histologique rare caractérisé par une croissance rapide avec infiltration locorégionale et un pronostic sombre. Nous rapportons l’observation d’un homme âgé de 63 ans exploré pour douleur de l’hypochondre droit évoluant depuis deux mois. La tomodensitométrie abdominale a objectivé une masse tissulaire du fond vésiculaire envahissant le foie. Une cholécystectomie a été réalisée. À l’examen histologique, il s’agissait d’un carcinome épidermoïde bien différencié et kératinisant.

Colorectal cancer in young adults: a retrospective study of 32 tunisian patients

Young people under the age of 40 with colorectal cancer represent a distinct subgroup with a more aggressive disease behaviour compared to older patients. This study aim to provide an updated overview on clinicopathological features, treatment and outcome of colorectal cancer in young adults under the age of 40. In our retrospective study, we reviewed 32 cases of colorectal cancer in young adults aged less than 40 years that were diagnosed at the pathology department of Mongi Slim hospital over a fifteen-year period (April 2000 - November 2014). Our study group included 13 male and 19 female patients (sex-ratio M/F = 0,68) between 17 and 39 years of age (mean = 31,25 years). The presenting clinical symptoms were dominated by altered bowel habits (n=17), followed by bleeding per rectum (n=16). Histopathological examination of the surgical and biopsy specimens established the diagnosis of mucinous adenocarcinoma in nine cases, well-differentiated adenocarcinoma in 11 cases, moderately differentiated adenocarcinoma in six cases, poorly differentiated adenocarcinoma in four cases and signet ring cell carcinoma in two cases. The tumours were classified after surgery as stage I (n = 2) (6%), stage IIA (n = 7) (22%), stage IIB (n=4) (13%), stage IIC (n=1) (3%), stage IIIB (n=8) (25%), stage IIIC (n= 4) (12%), stage IVA (n=4) (13%) and stage IVB (n=2) (6%). During the follow-up period which ranged between one month and 9 years, local recurrence of the tumour occurred in six cases, seven patients had hepatic metastases and seven patients died after a mean follow-up period of seven months. Molecular genetic studies are increasing the understanding of the pathobiology of colorectal cancer and may ultimately allow at-risk patients to be identified at an earlier stage.