Facteurs prédictifs de malignité des tumeurs oncocytaires de la thyroïde

INTRODUCTION : Les tumeurs oncocytaires (TO) de la thyroïde regroupent les adénomes et les carcinomes. La distinction entre malignité et bénignité constitue une difficulté de taille sur le plan histologique. De cet aspect découlent des contraintes dans l'attitude thérapeutique à adopter. L'objectif du travail était de déterminer les particularités cliniques, pathologiques et pronostiques des TO de la thyroïde et dégager les facteurs prédictifs de malignité. MÉTHODES : Etude descriptive, rétrospective portant sur 35 cas de TO de la thyroïde opérés entre 2007 et 2015. Les prélèvements ont été étudiés au service d'anatomopathologie. Une étude statistique a été réalisée à la recherche des facteurs prédictifs de mali¬gnité.RÉSULTATS : L'âge moyen des patients était de 53 ans. Le sex-ratio a été de 0,09. Le traitement était chirurgical dans tous les cas. L'examen anatomopathologique définitif a confirmé le diagnostic de 26 adénomes oncocytaires, six carcinomes papillaires on-cocytaires et trois carcinomes vésiculaires oncocytaires. La taille moyenne de la tumeur était de 38,1 mm (10-120). Une irathérapie post-opératoire a été proposée pour les carcinomes. En analyse univariée, les facteurs prédictifs de malignité étaient : l'âge supé¬rieur à 55 ans, la consistance dure, les limites floues, la double vascularisation anarchique, le caractère hypoéchogène du nodule, les microcalcifications, la colloïde sombre et dense, la capsule épaisse et la thyroïdite associée. En analyse multivariée, aucun de ces facteurs n'était prédictif de malignité.CONCLUSION : Les tumeurs oncocytaires de la thyroïde représentent une entité anatomo-clinique distincte. La détermination des facteurs prédictifs de malignité est essentielle pour adapter la prise en charge thérapeutique

Round cell sarcoma of the colon with CIC rearrangement.

BACKGROUND: The CIC-rearranged sarcoma is a very rare highly aggressive malignant soft tissue group of tumors. It has recently been described as highly aggressive soft tissue tumors of children and young adults sharing similar morphological features with the Ewing sarcoma. The digestive localization is exceptional. CASE PRESENTATION: A 14-year-old male presented with a history of abdominal pain for 1 year, which increased in intensity over the last 2 months. Imaging findings showed a large heterogeneous mesenteric mass on the left flank of the abdomen. Exploratory laparotomy was performed and revealed a large cystic hypervascularized mass depending on the transverse colon and mesocolon. A wide excision of the lesion was performed with segmental colectomy. No postoperative complications were noted. The microscopic examination revealed a vaguely nodular growth of undifferentiated small round cells, arranged in solid sheets separated by thin fibrous septa with a scarce stroma. After an uncomplicated post-operative course, the patient was referred for chemotherapy. The patient died 2 months later with a peritoneal and pleural progression. CONCLUSIONS: The CIC-rearranged sarcoma is an aggressive tumor. There is no standard therapy for this rare disease. Their treatment includes surgery and chemotherapy. Resistance to chemotherapy is common. Further publications and studies will help to determine a standard therapy for this rare disease.

[About a rare tumor of the kidney: Malignant mixed epithelial and stromal tumor]. / À propos d'une tumeur rarissime du rein: la tumeur mixte épithéliale et stromale maligne.

Mixed epithelial and stromal tumor of the kidney is a recently described neoplasm that predominantly affects perimenopausal women. Few cases with malignant features have been reported. Here, we report the case of malignant mixed epithelial and stromal tumor of the kidney with sarcomatous transformation arising in a 27-year-old female. She presented with abdominal discomfort. Computed tomography of the abdomen revealed a large compressive mass arisen from the left kidney with solid and focal cystic components. The patient underwent left radical nephrectomy. Histologic sections showed benign and malignant components. The benign component consisted of multiple tubules and variably sized cysts lined by benign epithelium. The malignant component was composed of undifferentiated cellular spindle cell sarcoma. By immunohistochemical studies, the epithelial component was positive for cytokeratins and epithelial membrane antigen (EMA). The stromal component displayed strong immunohistochemical expression of vimentin, CD99, bcl2; and was negative for cytokeratins, desmin, SMA, S-100, estrogen receptor (ER) and progesterone receptor (PR). Analysis by reverse transcriptase polymerase chain reaction (RT-PCR) failed to identify the SYT-SSX1 or SYT-SSX2 fusion transcripts characteristic of synovial sarcoma.

Multiple spinal metastases from a well-differentiated liposarcoma of the iliac wing: a case report.

STUDY DESIGN: A case report. OBJECTIVES: To report an unusual case of multiple spinal metastases from an undiagnosed well-differentiated liposarcoma (WDLPS) of the iliac wing and to stress the need of a meticulous clinical examination and further screening of patients with chronic and asymptomatic bony lesions. SETTING: University of medicine of Monastir, Department of neurological surgery, Fattouma Bourguiba University Hospital, Monastir, Tunisia and University of Medicine of Tunis EL Manar, Department of neurological surgery, Tunisian National Institute of Neurology, Tunis, Tunisia. METHODS: A 39-year-old man presented with signs of spinal cord compression for the past 2 weeks. His medical history was consistent for an asymptomatic right iliac wing mass that appeared 3 years ago and for which he has not consulted. Magnetic resonance imaging revealed multiple bony lesions of the thoraco-lumbar spine associated with a 6-cm right paravertebral mass at the T4 level extending posteriorly through the intervertebral foramina to the spinal canal causing major spinal cord compression. An emergent T2-T6 laminectomy allowed for a complete resection of the epidural mass. Pathological examination confirmed the diagnosis of well-differentiated liposarcoma. Adjunctive radiation therapy was administered. RESULTS: The patient's neurological status improved remarkably under an intensive care and rehabilitation program. He was ambulatory without assistance in the second postoperative week. CONCLUSION: The case reported in this paper represents a genuine example of the possible metastatic potential of WDLPSs of the bone and underscores the importance of examining patients thoroughly, especially when they have chronic and asymptomatic lesions.

[Paraganglioma of spermatic cord]. / Paragangliome du cordon spermatique.

Paragangliomas can exist anywhere in the distribution of neural crest derived cells. In the spermatic cord, they are exceedingly rare. We report a case of histologically discovery, occurring in a man of 50 years who had a para-testicular mass, painless, firm without other signs associated. In view of this observation and a review of the literature, we propose to establish the epidemiological profile and study the prognostic and the clinicopathologic features of this tumor.

[Primary cutaneous osteosarcoma]. / Ostéosarcome cutané primitif.

BACKGROUND: Osteosarcoma is a malignant tumor of mesenchymatous origin that chiefly affects the metaphysis of long bones. The extraskeletal form of the disease is rare, and even rarer is a cutaneous site, whether metastatic or primary. Herein, we report a new case of primary cutaneous osteosarcoma. PATIENTS AND METHODS: A 54-year-old woman presented a hard subcutaneous nodular tumor on her left arm noted 1 year earlier. The diagnosis of cutaneous osteosarcoma was made on the basis of histological analysis of the lesion, which showed a sarcomatous dermal-hypodermal proliferation secreting osteoid. Clinical and radiological staging ruled out any extra-cutaneous spread, particularly to bone, thus confirming the primary cutaneous nature of the osteosarcoma. DISCUSSION: Primary cutaneous osteosarcoma is a rare tumor, diagnosis of which is normally based on histopathological features.

[Sweet's syndrome: retrospective case series of 47 patients]. / Syndrome de Sweet : étude rétrospective de 47 cas.

PURPOSE: Also called acute febrile neutrophilic dermatosis, Sweet's syndrome is an inflammatory disorder with a prominent cutaneous expression. It is characterized by a variety of manifestations, clinical and histological findings. The objective of this study was to describe their clinical, pathological and therapeutic characteristics. METHODS: We report on a series of 47 patients who presented a Sweet's syndrome, collected in our institution in Tunis between 1997 and 2011. RESULTS: The patient population consisted of 11 men and 36 women. The mean age was 47 years with extreme ranging from 28 to 74 years. An associated disorder was observed in ten patients: inflammatory disease (three cases), inflammatory bowel disease (two cases), tuberculosis (three cases) and diabetes (three cases). One case of pregnancy was observed. Cutaneous lesions consisted of erythematous plaques or nodules. Lesions were located mainly on the upper or lower extremities. All biopsy specimens demonstrated a dermal infiltrate composed predominantly of neutrophils. Fibrinoid necrosis and intramural inflammation were observed in eight cases. CONCLUSION: The skin disorder can precede, follow, or appear concurrent with the diagnosis of an associated disease which requires careful surveillance.

Spindle cell oncocytoma of the adenohypophysis in a woman: a case report and review of the literature.

INTRODUCTION: Spindle cell oncocytoma of the adenohypophysis is a rare tumour recently reported by Roncaroli et al. in 2002. This tumour is considered a grade I tumour by the World Health Organization. CASE PRESENTATION: We describe what is, to the best of our knowledge, the 14th case of its kind in the literature. A 45-year-old African woman presented clinical and radiological findings related to a nonfunctioning pituitary adenoma. The diagnosis was made on the basis of histological and immunohistochemical findings. CONCLUSION: The purpose of this work is to report a rare pituitary tumour and to describe its histological and immunohistochemical features, which were characterized by the expression of thyroid transcription factor 1 antigen by tumour cells. This fact could support the theory of a possible common origin of these tumours in pituicytomas. In fact, thyroid transcription factor 1 is considered to be a specific marker of pituicytes.

[Central nervous system medulloepithelioma. A report of three cases]. / Le médulloépithéliome du système nerveux central. À propos de trois cas.

Central nervous system medulloepithelioma is a rare, highly malignant childhood tumor. It might be confused with medulloblastoma or other primitive neuroectodermal tumors, but it is quite particular by its clinical, radiological, and pathological features. The mean survival varies depending on whether or not a gross-total resection is possible. Adjuvant radiochemotherapy is often indicated. Only two reported cases in the literature survived beyond 4 years after treatment by gross total resection and radiotherapy without chemotherapy. We report three cases of supratentorial medulloepithelioma occurring in three children aged 11-17 years. Two patients underwent a gross-total resection followed by radiotherapy and survived more than 4 years after treatment. The third case had, however, recurred twice within the 1st postoperative month despite a complete resection each time and metastasis to the lung developed. Chemotherapy was then carried out after the third procedure and the patient died 7 months later.

[Paraparesis and fever in a Tunisian woman: cryptococcal spondylitis with spinal involvement]. / Paraparésie fébrile chez une Tunisienne: spondylite à cryptocoque avec atteinte médullaire.

Cryptococcus neoformans is a ubiquitous yeast that causes opportunistic infections mainly involving the central nervous system. Cryptococcoma is a rare entity characterized by a solid, tumor-like mass that is usually located in the cerebral hemispheres or cerebellum. Spinal involvement is rare with only 6 cases reported in literature. Bony involvement is also a rare occurrence that has been observed in only 5 to 10% of reported cases of infection by Cryptococcus neofomans. The purpose of this report is to describe a case of paraplegia due to cryptococcal spondylitis with spinal cord involvement in an HIV-seronegative patient with a history of systemic sarcoidosis. Diagnosis was achieved by histological examination of the surgical specimen.

Extraventricular neurocytoma in a child mimicking oligodendroglioma: a diagnostic pitfall.

Extraventricular neurocytomas are rare neuronal tumours that have been included in the 2007 WHO classification as a variant of central neurocytoma. They arise outside the ventricles, usually within the cerebral hemisphere,s but also in other regions throughout the neuraxis. The morphological overlap of these tumours with oligodendroglioma often poses diagnostic difficulty. Herein, a case of extraventricular neurocytoma in a 4-year-old girl is reported that mimicked histologically oligodendroglioma. The authors describe the clinicopathological features of this rare entity with special emphasis on differential diagnosis.

[Epidemiology of extrapulmonary hydatid cysts: 265 Tunisian cases]. / Epidémiologie des kystes hydatiques extrapulmonaires : 265 cas en Tunisie.

AIM OF STUDY: The authors wanted to analyze the epidemiological features of extrapulmonary hydatid cysts and compare their results with those reported in literature. METHODS: Two hundred and sixty-five cases of extrapulmonary hydatid cysts collected from 1990 to 2007 were retrospectively studied. RESULTS: One hundred and one male and 164 female patients (sex ratio M/F=0.61 mean age 38.7 years) were included. In our series, hydatid cysts involved mainly the kidney (24.1%), the central nervous system (22.6%), the liver (19.6%) and the spleen (11.3%). CONCLUSION: Contrary to published data, our results show that hydatid cysts of the kidney and of the central nervous system are more frequent than hepatic location which ranks 3rd. Such unusual results may be due to a selection bias.

Cutaneous gamma-delta T-cell lymphoma arising in the setting of Behcet's disease.

Cutaneous gamma-delta T-cell lymphoma (CGD-TCL) is an uncommon lymphoma composed of a clonal proliferation of mature activated gamma-delta T-cells expressing a cytotoxic phenotype. Malignant lymphoma is rarely associated with Behcet's disease, as only 12 cases have been reported in the literature, including a case of cutaneous T-cell lymphoma. In this report, the authors present a new case of CGD-TCL emerging in the course of Behcet's disease in a 40-year-old man. Diagnosis of CGD-TCL was established based on the combination of clinical, histological, immunophenotypical and molecular findings. Through a review of the current literature, the authors analyse the unique clinicopathological, molecular and immunohistochemical features of this rare cutaneous lymphoma.

Extragastrointestinal stromal tumor of the urinary wall bladder: case report and review of the literature.

Most mesenchymal tumours of the gastrointestinal tract are now referred to as gastrointestinal stromal tumours (GISTs). These tumours typically express c-kit (CD117) and CD34; 30-50% are (often focally) positive for alpha-smooth muscle actin, and all are negative for desmin and S100 protein. Recently, mutations in exon 11 of the c-kit gene have been identified as a molecular genetic marker for the subset of GISTs. In this report, we describe a mesenchymal tumour removed from the pelvic cavity of a 34-year-old man. The tumour was strongly attached to the external wall of the urinary bladder. The neoplasm grossly resembled a leiomyoma, and was histologically composed of sheets of spindle cells with a dense collagenous background. The mitotic activity was low (less then 1 per 50 high-power fields). Immunohistochemically, tumour cells were negative for alpha-smooth muscle actin and desmin and positive for CD117 and CD34. This case illustrates that tumours which are phenotypically and genotypically similar to GISTs may present in sites other than the tubular gastrointestinal tract.

Renal epithelioid angiomyolipoma: a case report and literature review.

BACKGROUND: Renal epithelioid angiomyolipoma is a recently recognized variant of angiomyolipoma, closely simulating renal cell carcinoma both clinically and histologically. Only a relatively small number of cases of epithelioid angiomyolipoma of the kidney have been reported. AIM: To highlight clinicopathological features of this rare tumour. OBSERVATION: We report herein a new case of renal epithelioid angiomyolipoma in a 38-year-old male with no stigmata of tuberous sclerosis. The tumour was composed of diffuse sheets of epithelioid cells, small numbers of adipocytes and occasional blood vessels. Immunohistochemically, neoplastic cells were immunoreactive for HMB-45, but negative for cytokeratin. The patient showed no evidence of recurrence or metastatic disease one year after radical nephrectomy. CONCLUSIONS: Epithelioid angiomyolipoma may be locally aggressive and can metastasise; therefore, long-term post-operative follow-up is mandatory.

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.

Subependymomas: a clinicopathological study of 6 symptomatic cases.

BACKGROUND: Subependymomas are rare, slow-growing, ependymal neoplasms that commonly occur in the fourth or lateral ventricles. OBJECTIVE AND DESIGN: A retrospective study of 6 histologically proven subependymomas was undertaken to analyse their clinicopathological characteristics. RESULTS: There were five male and one female patients ranging in age from 11 to 50 years (mean 35.8 years). All patients were symptomatic at diagnosis. The most common clinical presentations included headache (n=6) and vomiting (n=3). Tumours were located in the lateral ventricle in five cases and in the fourth ventricle in one case. Magnetic resonance imaging detected obstructive hydrocephalus in all cases. Five patients underwent gross total resection and one patient had subtotal excision of the tumour. Histologically, all tumours were characterised by clustering of isomorphic cells arranged against a fibrillary background. Focal cystic degeneration was seen in 5 tumours. During the follow-up period, which ranged between 2 months and 10 years, all patients were symptom-free with no evidence of recurrence.

Calcified pilocytic astrocytoma of the medulla mimicking a brainstem "stone".

Brainstem gliomas are a heterogeneous group of tumours commonly found in children, comprising about 10% of central nervous system tumours in paediatric patients, but less than 2% in adults. Pilocytic astrocytomas usually involve the midbrain and the medulla, and their surgical resection, when feasible, is generally curative. Thin calcifications can be normally found within low grade gliomas, but densely calcified pilocytic astrocytomas of the brainstem have been only rarely reported. We present the case of a young man presenting with a large brainstem calcification involving the medulla, which was subtotally resected using a posterior suboccipital approach. The definitive pathological diagnosis was calcified pilocytic astrocytoma.