Slim Haouet

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.

Solitary fibrous tumour of the central nervous system: pathological study of eight cases and review of the literature

Aims: Solitary fibrous tumours (SFT) of the central nervous system are rare neoplasms that usually present as dura‐based masses and clinically resemble meningiomas. Histologically, they can be similar to haemangiopericytoma or fibrous meningioma. We present herewith a pathological study of eight patients with SFT and compare our results with the published data. Methods: All tumours diagnosed as SFT over a period of 15 years were retrieved from the files of La Rabta Hospital Department of Pathology, Tunisia. Routine histological sections were prepared from formalin‐fixed, paraffin‐embedded specimens. Immunohistochemical analysis was performed using antibodies raised against vimentin, CD34, CD99, Bcl‐2 and MIB‐1. Results: The SFT group consisted of four males and four females with a mean age of 44 years. The tumours were supratentorial in six cases, tentorial in one case and located in the lateral right ventricle in the last case. All patients underwent surgery as initial treatment. Histologically, SFT were similar to their soft tissue counterparts. Two cases demonstrated increased cellularity, marked nuclear hyperchromasia and marked cellular pleomorphism. Only the later two cases recurred after 6 months of follow‐up. Conclusion: Little is known about the biological behaviour of SFT, although most seem amenable to total resection.

Descriptive Epidemiology of Childhood Central Nervous System Tumours in Tunisia

Introduction: Central nervous system tumours represent 20% of all childhood cancers, and are the second most common group of neoplasms after leukaemias. Objective: To describe epidemiological characteristics of central nervous system tumours in a paediatric Tunisian population. Patients and Methods: A retrospective study of 492 childhood central nervous system tumours operated between 1990 and 2004 was undertaken. We investigated the age-related location, gender distribution and the histology of all tumours, and adopted the latest WHO classification (2007) in grouping all the tumours. Results: There were 488 primary and 4 secondary tumours; 426 (86.6%) were intracranial and 66 (13.4%) were intraspinal. Of the 426 intracranial tumours, 214 (50.24%) were supratentorial and 212 (49.76%) were infratentorial. The median age at diagnosis was 8 years, with a male:female ratio of 1.14:1. Low-grade tumours (WHO I/II) constituted 67.3% of all lesions and the rest (32.7%) were high-grade tumours (WHO III/IV). The most common tumour found in our series was astrocytoma (38%), followed by medulloblastoma (16.2%), then ependymoma (6.9%), cystic tumours (6.3%) and craniopharyngioma (5.3%). The overall 5-year survival rate was 45% with a mean follow-up period of 36 months. Conclusion: In our patient population, the incidence and distribution of central nervous system tumours were similar to those reported in literature. Overall survival rates varied according to tumour location and histopathology.

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.

Gangliogliome malin cérébelleux : À propos d'une observation, avec revue de la littérature

Resume Le gangliogliome (GG) est une tumeur nerveuse, habituellement bien differenciee, de croissance lente, composee de cellules neuroganglionnaires matures melees a une composante gliale de grade I ou II et beaucoup plus rarement anaplasique de grade III. Uniquement 12 cas de gangliogliomes recemment publies presentent une composante gliale manifestement maligne a type de glioblastome et sont consideres, selon la classification de l’OMS, de grade IV. Nous en rapportons un cas survenu chez une femme âgee de 25 ans aux antecedents familiaux de neurofibromatose de type I. Ce gangliogliome de grade IV se distinguait par un contingent glial repondant a un glioblastome multiforme et dont la richesse en cellules geantes masquait l’existence de cellules neuroganglionnaires. Un tel aspect a necessite un examen immunohistochimique afin de distinguer les deux populations cellulaires. Notre observation constituerait le second cas de gangliogliome malin grade IV ou le suivi est precise jusqu’au deces du patient. Nous discuterons son diagnostic differentiel et nous exposerons son profil clinique ainsi que son pronostic.Ganglioglioma is usually a well differentiated slowly growing mixed neuronal and glial neoplasm corresponding to WHO grade I or II. However, some gangliogliomas are considered to be WHO grade III because they exhibit anaplastic features in their glial component. Finally there are exceptionally rare cases of newly diagnosed gangliogliomas with grade IV changes in the glial component. We report a case of a 25-year-old woman with a family history of neurofibromatosis who presented initially with a World Health Organization grade IV anaplastic ganglioglioma (a mixed ganglion cell multiform tumor glioblastoma). Despite aggressive management, the patient died of disease in a relatively short period. Histologically, two cell populations were noted: a predominant glial component consisting in a multiform glioblastoma and ganglion cells supporting a diagnosis of ganglioglioma. Immunohistochemical analysis clearly distinguished the two tumor cell populations. Although other cases of grade III gangliogliomas and twelve cases of grade IV gangliogliomas have been reported, the present case is exceptional in that, to our knowledge, it is the second case of a patient who presented initially with a composite grade IV ganglioglioma and who was clinically followed up to the time of death. This case allows direct comparison between the histological findings in a multiform glioblastoma and a ganglioglioma. It also documents the aggressive biologic behavior of this complex neoplasm.

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.

Hemangiopericytoma in the central nervous system. A study of eight cases.

Most hemangiopericytomas (HPC) are located in the musculoskeletal system and the skin, while the location in the central nervous system (CNS) is rare. The latter represents 2 to 4% in large series of meningeal tumors, thus accounting for less than 1% of all CNS tumors. In the central nervous system, tumors with a hemangiopericytomatous histolopathological pattern can be either hemangiopericytomas or solitary fibrous tumors. CNS-HPCs have a relentless tendency for local recurrence and metastases outside the CNS. Metastasis can also appear many years after adequate treatment of the primary tumor. We present a pathological study of eight patients with CNS-HPC and compare our results with corresponding published data. The CNS-HPC group consisted of three males and five females with a mean age of 36.75 years. The tumors were supratentorial in four cases, infratentorial in two cases, tentorial in one case and located in the spinal cord in the last one. Histologically, CNS-HPCs were similar to their soft tissue counterparts. One case demonstrated increased cellularity, marked nuclear hyperchromasia and marked cellular pleomorphism with infiltration of the cerebellum. All patients underwent surgery with gross-total resection in all cases. No patients received postoperative radiation therapy. Only four patients recurred locally after six, seven and eight months, and five years. Our study presents the pathological features of CNS-HPC as a distinct entity from both meningioma and solitary fibrous tumors. A comparative review of literature with our results is discussed.

Synovial fluid and serum levels of IL-17, IL-23, and CCL-20 in rheumatoid arthritis and psoriatic arthritis: a Tunisian cross-sectional study

Sir, Rheumatoid arthritis (RA) is a chronic inflammatory autoimmune disease characterized by abnormal synovial hyperplasia associated with local infiltration of various inflammatory cells leading to cartilage and bone destruction. Interleukine-17-producing T helper (Th17) cells are a recently discovered effectors T-lymphocyte subset that plays a critical role in several chronic inflammatory diseases, like RA and psoriatic arthritis (PsA) [1–4]. The objective of our study was to appreciate the role of interleukine (IL)-17, IL-23, the major Th17 driving cytokine, and CCL-20, a major Th17-attracting chemokine in the regulation of RA and PsA, by comparing concentrations in sera and in knee synovial fluid (SF) among three groups of patients, one with an active form of RA, one with active peripheral psoriatic arthritis PsA and a control group of osteoarthritis (OA) (Table 1). A case–control study was carried out, during the period of January to May 2010, in the department of Rheumatology with the Immunology laboratory of La Rabta hospital at Tunis (Tunisia). The first group of 26 patients with active form of RA (DAS 28 [ 5.1), according to the 2010 ACR/ EULAR criteria [5], was composed of 21 women and 5 men, with a mean age of 46 ± 12 years and a mean disease duration of 13.5 ± 6 months. The second group was composed of 18 patients with active peripheral PsA, fulfilling the CASPAR criteria [6], with a mean age of 35 ± 9 years and mean disease duration of 20 ± 7 months. The control group was composed of nine patients with flare-up of OA, with a mean age of 56.5 ± 12 years and a mean disease duration of 36 months ± 9. All patients were enrolled after informed consent. Paired concentrations of IL-17, IL-23, and CCL-20 in sera and in SF were determined using an enzyme-linked immuno-sorbent assay (ELISA) technique (R&B, United States). A correlation analysis was performed between parameters of RA’s activity (number of tenderness joints, number of swollen joints, number of night awakening, duration of morning stiffness, disease activity score DAS 28, modified Sharp score [7], serum and SF levels of all the mediators (IL-17, IL-23, and CCL-20). Analyses were performed using SPSS software. Non-parametric tests were used to compare the concentrations, Kruskal–Wallis and Mann–Whitney tests for the comparison of independent samples and paired Wilcoxon test for the comparison of paired samples. Correlations were calculated using the Spearman’s coefficient. A P value below 0.05 was considered as significant. The IL-17 and IL-23 levels were similar in the serum of patients with RA, PsA, and OA. This finding was also demonstrated in the joints of patients with RA, PsA, and OA. In addition, only the synovial concentrations of CCL20 were significantly higher in RA patients than the control groups (P = 0.001). However, when comparing the SF CCL-20 levels in RA versus PsA, no significant difference was found. D. Mrabet (&) S. Sellami Department of Rheumatology, La Rabta Hospital, 1007 Tunis, Tunisia e-mail: mrabetdalila@yahoo.fr

Congenital salivary gland anlage tumor of the nasopharynx.

Nasal and upper respiratory tract obstruction in the neonatal period can result from a variety of conditions, and may be present with variable symptoms. Salivary gland anlage tumor, also referred as congenital pleomorphic adenoma, is a very rare benign congenital tumor of the nasopharynx, which may produce nasal obstruction and other associated, nonspecific symptoms. We report a case of congenital salivary gland anlage tumor causing a severe neonatal respiratory distress with pulmonary hypertension. The tumor was removed and the outcome was favourable without recurrence at five years of the follow up.

Multiple myeloma and cutaneous anaplastic large T-cell lymphoma in the same patient: is there a causal relation?

Synchronous occurrence of lymphomatous proliferations of B and T lineage in the same patient is a very rare event and still poorly understood. All the cases reported in the English language literature are described as single case reports.