ALK-Negative Inflammatory Myofibroblastic Tumor: A Challenging Case in a Premature Newborn.

Inflammatory myofibroblastic tumors (IMT) are rare borderline tumors with a variable histological appearance that may mimic multiple mesenchymal tumors. We present a rare case of a challenging abdominal mass discovered in a premature newborn. The histopathology showed a bland myofibroblastic proliferation associated with an inflammatory infiltrate that was positive for smooth muscle actin and desmin but negative for anaplastic lymphoma kinase (ALK) protein. The diagnosis of an ALK-negative IMT was established. The tumor was partially resected. After six months of follow-up, the residual tumor remained stable, and the patient was asymptomatic. The correct diagnosis and subsequent treatment of ALK-negative IMT require appropriate histopathological, immunohistochemical, and sometimes genetic examination. Further research has to be conducted to help clinicians make an appropriate treatment plan.

46,XX male syndrome with mullerianosis of the urinary bladder: A new clinical entity.

Müllerianosis of the urinary bladder is a rare entity characterized by the presence of an admixture of at least two types of müllerian tissue in the muscularis propria of the bladder. We report the case of a 43-year-old male patient presented for urological evaluation due to episodes of total gross hematuria. Physical examination revealed ambiguous external genitalia. Abdomiopelvic CT scan revealed a tumor at the level of the bladder floor. Transurethral resection of the bladder lesions was done and histopathological studie confirmed the diagnosis of müllerianosis. Karyotype analysis showed a 46 XX male syndrome.

Buschke - Lowenstein anal tumor: an ambiguous entity.

The Buschke - Lowenstein tumor is a rare sexually transmitted disease. Its location at the anal margin is also very rare. The most incriminated risk factor is human papillomavirus infection. Its clinical form may be confusing with other tumor and infectious lesions. Histologically, it is characterized by a well-differentiated malpighian proliferation. It represents local aggressive behavior. The treatment of reference remains the surgery with healthy margins of excision. Other treatments have been tested, but their effectiveness remains uncertain. We report here a new case of anal margin Buschke - Lowenstein tumor with a review of the literature.

Pediatric high grade gliomas: Clinico-pathological profile, therapeutic approaches and factors affecting overall survival.

INTRODUCTION: Pediatric high grade gliomas are rare tumors of the central nervous system. Treatment is multidisciplinary, comprising surgical excision followed by radiotherapy and/or chemotherapy. OBJECTIVES: describe these tumors' characteristics as seen in our institution, and identify factors associated with better overall survival. PATIENTS AND METHODS: We conducted a retrospective study of 30 cases of pediatric high grade glioma treated consecutively in our institution over a 20-year period. Brainstem tumors and patients aged more than 22years were excluded. Univariate analysis was conducted to determine factors associated with better overall survival. RESULTS: The series comprised 30 pediatric high grade gliomas: 27 glioblastomas and 3 anaplastic astrocytomas. The sex ratio was 1.7. Mean age was 13years. Tumors were mainly located in the cerebral hemispheres (63.3%). Median tumor size was 5cm. Glioblastomas were subdivided into 26 cases of classical subtype (96.3%) and 1 case of epithelioid subtype (3.7%). Surgical strategy consisted in tumor resection in 24 cases (80%). Twenty-one patients (70%) received postoperative radiotherapy. Therapeutic response at end of treatment was complete in 7 cases (23.3%). Postoperative radiation therapy and complete treatment response were significantly associated with improved overall survival in all high grade gliomas and also specifically in glioblastomas (P<0.001 and P=0.005, respectively). CONCLUSION: Our results suggest that postoperative radiotherapy and complete treatment response are predictive factors for better overall survival in pediatric high grade glioma.

Well-differentiated extraskeletal chondrosarcoma: about a new case.

Extraskeletal chondrosarcoma is a rare malignant tumor. The well differentiated histological type, which is found primary in soft tissue, is extremely rare. This report  describes the case of a 58-year-old woman presented with a large palpable mass in the right buttock. Imaging studies revealed a well-defined soft tissue mass, with extensive calcification. A histological examination after surgical resection confirmed the diagnosis of well-differentiated extraosseous chondrosarcoma. The outcome was favorable, without recurrence or metastasis.

Gastric signet-ring cell carcinoma with hypersecretion of ß-Human chorionic gonadotropin and review of the literature.

ß-Human chorionic gonadotropin (ß-HCG) is an embryonic protein secreted by the syncytiotrophoblast of the placenta. The determination of the plasma ß-HCG level is routinely used for the diagnosis and the follow-up of germ cell tumors. Some adenocarcinomas have been described as being rarely associated with ß-HCG hypersecretion. We report a case of gastric signet-ring cell carcinoma with ß-HCG hypersecretion and propose hypotheses to explain the pathogenesis of such hypersecretion.

[Isolated sternal tuberculosis in immunocompétent adult]. / Tuberculose sternale isolée chez l'adulte immunocompétent.

Isolated sternal tuberculosis is a rarely described entity even in countries where tuberculosis is endemic. We report the case of 25 old years patient who presented with a chest wall mass. Imaging concluded to a (ring-enhancing hypodense soft tissue mass surrounding the sternum with sternal fracture). Malignancy was eliminated by a core needle biopsy. We noted clinical and radiological recovery with medical tuberculosis treatment. Neoplastic origin was removed by biopsy and anatomopathological study of the lesion.

Microcystic transitional cell carcinoma: a rare tumor of the urinary bladder.

Microcystic urothelial carcinoma is a rare variant of invasive transitional cell carcinoma recognized by the WHO classification. It is characterized by its deceptively benign appearance. The clinical course of this uncommon variety of carcinoma is not well known and their histological and immunohistological features are not well defined. We report a case of a 37-year-old man with a microcystic transitional cell carcinoma of the urinary bladder. He was diagnosed 4 years ago with cystitis glandularis lesions and nephrogenic adenoma. Through this observation we will try to define the clinical and pathological features of this uncommon tumor which must be differentiated from a number of proliferative lesions of the urothelium. The poor prognosis and aggressiveness of this tumor seems to be related to a higher stage and grade at diagnosis.

[Parathyromatosis: An uncommon cause of recurrent hyperparathyroidism]. / Parathyromatose : une cause rare d'hyperparathyroïdie récidivante.

INTRODUCTION: Parathyromatosis is a rare cause of recurrent hyperparathyroidism. It results from hyperfunctioning parathyroid tissue scattered throughout the thyroid bed region. CASE REPORT: A 51-year-old man with a history of parathyroidectomy, presented 18 years later with recurrent primary hyperparathyroidism. Surgical exploration identified a single parathyroid gland. The act was completed by a central compartment dissection and ipsilateral lobectomy. The patient was free of recurrence after a one-year follow-up. CONCLUSION: Parathyromatosis a rare cause of recurrent hyperparathyroidism. Its management is challenging. Extensive surgery is required with clearance of the central neck compartment and homolateral lobectomy. Medical therapy could be used to decrease parathormone level in recurrent parathyromatosis.

[Neuroendocrine carcinoma of the bladder: A single centre retrospective study]. / Carcinome neuroendocrine de la vessie: étude rétrospective monocentrique.

PURPOSE: Neuroendocrine carcinoma of the bladder is a rare tumour representing 0.5 to 1% of bladder tumours. It is a specific histological entity characterized by rapid metastatic dissemination and poor prognosis. The aim of this study was to describe the epidemiological, clinical, therapeutic modalities and the evolutive aspects of patients receiving a treatment for bladder neuroendocrine carcinoma. PATIENTS AND METHODS: Between January 2004 and January 2014, seven patients received a treatment for a neuroendocrine carcinoma of the bladder, at the department of oncology, Habib-Bourguiba Hospital, in Sfax, Tunisia. RESULTS: The median age was 58 years. All patients were male. Neuroendocrine carcinoma was pure in four cases and associated with urothelial carcinoma in the other three cases. Two patients were diagnosed at a metastatic stage. A cystectomy was performed in two cases. One patient received a chemotherapy and radiotherapy. The other four patients received chemotherapy alone. A single case of complete remission was observed. Median survival was 15 months (5-30 months). One patient is still alive 30 months after diagnosis. CONCLUSION: The management of neuroendocrine carcinoma of the bladder is not standardized and requires a multidisciplinary consultation.

Telangiectatic osteosarcoma of the rib: a rare entity and a potential diagnostic pitfall.

Osteosarcoma (OS) is a common primary malignant tumor of bones that produces osteoid matrix. Telangiectatic osteosarcoma (TOS) is a rare variant of OS. It affects the long bones especially the lower end of femur and the upper ends of tibia and humerus, a distribution similar to the conventional osteosarcoma. The rib involvement is very infrequent. We present a case of TOS of the rib that posed a diagnostic difficulty owing to its unusual location and to its resemblance to giant cell tumor and aneurysmal bone cyst. Correspondence.

Uterine tumour resembling ovarian sex cord tumours presenting as multiple endometrial and cervical uterine polyps: a case report.

BACKGROUND: Uterine tumours resembling ovarian sex-cord tumours (UTROSCT) are very rare, benign uterine tumours, composed solely of sex cord elements. These tumours have a polyphenotypic immunophentype that favours a derivation from uterine mesenchymal stem cells. CASE REPORT: A 43-year-old female presented with recurrent vaginal bleeding. On hysteroscopy, she had multiple endometrial and cervical polyps that were removed endoscopically. Histologically, the specimen contained epithelioid cells arranged in tubules, trabeculae and anastomosing cords, without significant cellular atypia or mitotic activity. Immunohistochemical studies were performed. The tumour was found to be diffusely positive for vimentin, calretinin and desmin, focally positive for cytokeratin, CD99 and inhibin and negative for chromogranin and CD10. A subsequent total hysterectomy was performed and revealed neoplastic infiltration of the myometrium. CONCLUSION: A polyphenotypic immunophenotype is a characteristic feature of UTROSCT, and may be helpful in diagnosis and in exclusion of other lesions. Familiarity with this tumour by gynaecologists and pathologists is essential to avoid misdiagnosis:correct diagnosis of this neoplasm is important in patient management.

[Paraneoplastic cerebellar degeneration as the presenting manifestation of breast carcinoma: a case report]. / Dégénérescence cérébelleuse paranéoplasique révélant un cancer du sein : à propos d'un cas.

INTRODUCTION: Paraneoplastic cerebellar degeneration may be a manifestation indicative of lung, gynecological or breast cancer. Nevertheless, breast cancer is rarely revealed by the occurrence of a paraneoplastic syndrome. CASE REPORT: We report a 38-year-old patient who presented a paraneoplastic cerebellar degeneration with anti-Yo antibodies as the presenting manifestation of a breast cancer. CONCLUSION: The diagnosis of a paraneoplastic neurological syndrome (PNS) should lead to urgent and comprehensive screening for cancer oriented by the type of PNS and the nature of the anti-neuronal antibody.

Primary mucinous carcinoma of the thyroid gland: case report with review of the literature.

Primary mucinous thyroid carcinoma (PMTC) are extremely rare lesions that are histologically indistinguishable from mucinous carcinoma of other sites. We describe the clinicopathological, histological and immunohistochemical features of this rare tumour with a review of the literature. We describe a case of thyroid tumour, in 56-year-old Tunisian man, composed of small nests and sheets of malignant epithelial cells associated with extensive extracellular mucin that entrapped the follicular parenchyma of thyroid. Thyroglobulin and thyroid-specific-transcription factor 1 (TTFl) were focally positive. Follow-up did not reveal another neoplasm at other sites. Based on these features, we classified this tumour as PMTC. Mucinous carcinoma of the thyroid gland can be a cause of pitfall in differential diagnosis. For correct diagnosis, complete clinical history, restricted histological criteria and immunohistochemical panel are necessary.

Mammary myofibroblastoma with leiomyomatous differentiation: case report and literature review.

INTRODUCTION: Myofibroblastoma of the breast (MFB) is an unusual benign tumour that belongs to the family of benign spindle cell tumours of the mammary stroma. The detection of smooth muscle cells in MFB is explained by its histogenesis from CD34+ fibroblasts of mammary stroma capable of multidirectional mesenchymal differentiation, including smooth muscle. AIMS: The purpose of this case is to highlight characteristics of this rare neoplasm. Immunohistochemical features, in MFB with predominant leiomyomatous differentiation, are provided to offer a practical approach to a correct diagnosis. CASE REPORT: We report a right MFB in a 60-year-old male. The tumour was unusual due to its morphological features, with predominant leiomyomatous differentiation. Immunohistochemical findings, based on the negativity of h-caldesmon, helped in reaching a diagnosis. CONCLUSION: The detection of leiomyomatous rather than myofibrolastic features in MFB may reflect only the predominant cell types of examined area, and this is not necessarily representative of the remaining tumour which may have a different basic cellular composition. Immunohistochemical expression of h-caldesmon is a reliable marker in distinguishing smooth muscle versus myofibrolastic cellular differentiation in spindle cells lesions of the breast.

Gastric metastases. An endoscopic series of ten cases.

We report a series of ten cases of the clinical, endoscopic and pathological features of gastric metastases. Patients were six women and four men between 54 and 88 years old, with gastric metastases from breast carcinoma (4), lung carcinoma (4) and melanoma (2). Patients underwent an upper gastrointestinal endoscopy for epigastralgia (2), hematemesis (2), dysphagia (1) and anemia (5). On endoscopy, tumors appeared as nodules with a central ulceration (5), an ulceration (4) or simulating linitis plastica (1). Metastases were located in the cardia (2), fundus (5) and antrum (3). Primary tumors had been diagnosed between one day and 20 years before upper endoscopy. Eight patients had multivisceral metastases. The microscopic features of the gastric metastases resembled a primary gastric cancer in eight cases. Thanks to clinical data, the pathologist confirmed the diagnosis of gastric metastases on immunohistochemistry. Nine patients died in the eight-month follow-up period. Gastric metastases are rare, occur at a late stage of the neoplastic disease, and have a poor prognosis. Diagnosis of gastric metastases is difficult because they simulate primary gastric cancer on endoscopy and on microscopic examination. A correct diagnosis is based on good communication between gastroenterologists and pathologists.

[North African and Southeast Asian nasopharyngeal carcinomas: between the resemblance and the dissemblance]. / Le carcinome nasopharyngé en Afrique du Nord et dans le Sud-Est asiatique: entre similitude et différence.

Nasopharyngeal carcinoma (NPC) is an unusual head and neck cancer because of its unequal geographical distribution and its consistent association with the Epstein-Barr virus (EBV). This malignant tumor poses a serious public health problem in many countries, especially in Southeast Asia and North Africa where the recorded incidence are highest. During the past decade, a growing number of studies were undertaken to define the molecular basis of NPC. However, the analysis of several clinical and biological parameters of North African and Southeast Asian NPCs has shown notable differences, suggesting that they could result from a distinct combination of etiological factors. One intriguing characteristic of North African NPC, concerns its bimodal age distribution with a secondary peak of incidence in the range of 15-25 years, not observed in Asian NPC. In this juvenile form of NPC, immuno-histochemistry assay has shown that the two key proteins controlling the apoptotic-survival balance p53 and Bcl-2 are less frequently expressed whereas the transmembrane tyrosine-kinase receptor c-kit and the main EBV oncoprotein LMP1 were more abundant. In addition, the EBV serological alterations are less informative for the diagnosis of the juvenile compared to the adult form. In addition, most North African NPCs contain EBV strains with genetic polymorphisms distinct from those described in the Southeast Asia series (predominance of F, D, H1-H2, XhoI+ and f, C, H, XhoI- respectively). In contrast, studies relating on tumor chromosomal alterations or aberrant promoter methylation result in data very similar to those obtained from the Southeast Asia series, supporting the concept of a common molecular basis for all NPC regardless of patient geographic origin.

[Eosinophilic dermatosis associated with hematological disorders: A clinical, histopathological and immunohistochemical study of six observations]. / Dermatose éosinophilique associée aux hémopathies : étude clinique, histopathologique et immunohistochimique de six cas.

BACKGROUND: Eosinophilic dermatosis of hematologic disease (EDH) or insect bite-like reaction is a pruritic dermatitis described mostly in patients with chronic lymphocytic leukaemia (CLL). We describe six patients with the disorder in association with CLL and other blood dyscrasias. PATIENTS AND METHODS: We reviewed the medical records of patients with EDH seen between 2004 and 2009 in our department and re-examined histological slides. RESULTS: Mean age at dermatosis onset was 75.6 years and the sex ratio was 1. There were three CLL, two mantle-cell lymphomas and one MALT-type lymphoma. The dermatitis was quite polymorphic, with erythematous papules, wheals and plaques. The initial skin lesions appeared at the same time as or after the diagnosis of haematological neoplasm. Their reappearance heralded relapse of the blood disease in three cases. Histologically, all lesions had a dense dermal infiltrate of small, mostly CD4+ T-cells, with numerous eosinophils. In three patients, there was marked folliculotropism, resembling folliculotropic T-cell lymphoma. In most cases, EDH disappeared after appropriate chemotherapy for the blood disorder. DISCUSSION: Our cases show that the clinical expression of EDH is quite polymorphic. Its appearance may precede relapse of or may indicate prompt search screening for blood dyscrasia. The most efficient treatment of this dermatosis appears to be specific chemotherapy for the blood dyscrasia. There is reason to believe that a population of T-helper 2 (Th2) lymphocytes, reactive to malignant B-cells, induces tissue eosinophilia, mainly through production of interleukin (IL)-5, among other cytokines. Eosinophils appear to be the main effector cells.