A rare case of eosinophilic jejunitis: diagnosis and management strategies.

Eosinophilic gastroenteritis is a rare disease with an unknown cause, which can manifest independently or as part of a hyper-eosinophilic syndrome. The severity of the condition depends on the extent of eosinophilic infiltration and damage to the digestive tract. Diagnosis relies on histological examination, which reveals a significant presence of eosinophilic polymorphonuclear leukocytes in the digestive wall. The authors present a new case of eosinophilic gastroenteritis in a 28-year-old patient who exhibited obstructive symptoms but lacked peripheral eosinophilia. Esophagogastroduodenoscopy showed no abnormalities, but barium transit imaging revealed gastro-duodeno-jejunal dilation upstream of a tight jejunal stenosis. Surgical examination of the affected area confirmed a diffuse and transparietal eosinophilic infiltrate, with no evidence of parasitic or granulomatous lesions. Fortunately, the patient had a swift recovery following surgery. Biopsies conducted at other locations, including the gastric, hepatic, and medullary levels, produced negative results, indicating the localized nature of the condition.

[Biphasic sarcomatoid carcinoma of the thyroid: an exceptional localization of a rare tumor]. / Carcinome sarcomatoïde biphasique de la thyroïde: localisation exceptionnelle d'une tumeur rare.

Thyroid sarcomatoid carcinoma is a rare and aggressive neoplasm composed of a follicular carcinoma which is contiguous or admixed with a pleomorphic spindle cell component. We report the case of a thyroid tumor reputed to have a poor outcome, in a 62-year-old woman. The radical thyroidectomy specimen was totally invaded. Results of immunoperoxidase staining for thyroglobulin and epithelial markers were positive in the areas of follicular carcinoma and negative in the sarcomatous component. The patient died a few days later due to septic shock. The epithelial and mesenchymal components of thyroid carcinosarcoma were both part of the neoplastic parenchyma and evolved from a single common stem cell, in agreement with the hypothesis that the tumors are of monoclonal origin. The definition of this tumor as its histogenesis and prognostic are discussed.

[An unusual localization of a rare tumor: giant-cell tumor of the rib. Case report and review of the literature]. / Une localisation inhabituelle d'une pathologie rare: la tumeur costale à cellules géantes. A propos d'un cas avec revue de la littérature.

INTRODUCTION: Giant-cell tumors of bone are rare primary neoplasms commonly encountered in young adults. Women are slightly more affected than men. CASE REPORT: We report the case a 27 year old woman presenting with a twelve months history of painful and progressively growing thoracic mass in the right anterior chest wall. Physical examination found out a fixed thoracic mass in the right retro-mammary area measuring 8 x 6 centimeters. The overlying skin was normal. Chest roentgenogram demonstrated a large ill defined mass continuing the anterior arc of the fourth right rib. Computed tomography evidenced a well defined pathologic process originating from the fourth right rib without expansion of the surrounding soft tissue. Pulmonary functional tests were normal and other complementary investigations evidenced no abnormalities. Our patient first had a fine needle cytological biopsy that brought strong suspicion of Giant-cell tumor of the rib. She then underwent an "en bloc" resection of the tumor whose histopathologic analysis allowed a definitive diagnosis. The post-surgical follow up during 12 months showed no signs of tumor recurrence. CONCLUSION: Through this observation the authors emphasize not only the rarity of the giant-cell tumors of bone but also its unusual costal localization (few cases reported till date). They focus on the importance of precocious screening and treatment and underline the value of the follow up in order to detect timely any sign of local recurrence or sarcomatous transformation. Finally, they report a current review of the literature.

[Intimal sarcoma of the inferior vena cava]. / Sarcome intimal de la veine cave inférieure.

INTRODUCTION: Primary sarcomas of the great vessels, that is, the aorta, pulmonary artery, and inferior vena cava, are rare. They can be classified according to the location of the sarcoma in the vessel wall and by their gross appearance. Most often they are leiomyosarcomas or fibrosarcomas. CASE: We report here a case of an intimal sarcoma of the inferior vena cava. Histological and immunohistochemical findings confirmed the diagnosis for this 17-year-old girl and distinguished it from leiomyosarcoma and angiosarcoma, both of which have better prognoses. DISCUSSION: Intimal sarcoma of the inferior vena cava is rare and difficult to diagnose before surgery or biopsy. Histologically, it is a poorly differentiated tumor with the worst prognosis among the primary vascular sarcomas. Pathologic findings and immunohistochemical staining are useful for a positive diagnosis.

Central nervous system tumors in Morocco. Retrospective analysis of 2374 cases.

BACKGROUND: No epidemiological data about central nervous system tumors in Morroco have been published. The objective of the present study is to assess topographic and demographic patterns of a large series of histologically confirmed tumors of central nervous system (CNS), skull and vertebral column, examined in the main neuropathology laboratory of the country. METHODS: No. 2363 tumors have been collected in the department of Neuropathology in Hôpital des spécialités oto-neuro-ophtalmiques, Rabat, between 1988 and 1997. Data concerning age, sex, tumor location and histological type were retrieved from the laboratory files. Histological typing was based on the World Health Organization (WHO) classification of central nervous system tumors and grading on the Kernohan and Mayo Clinic-St. Anne systems. RESULTS: No. 1454 intraparenchymatous and 904 extraparenchymatous tumors were studied. The topographic repartition of these tumors presented no differences when compared to the literature data. The main differences were noted for malignant astrocytomas and glioblastomas which were less frequent than in Western countries and occurred in younger people. The young age of Morrocan population probably explains these findings. Childhood tumors repartition was similar to Western series. CONCLUSIONS: These results emphazise the need for a population based registry in order to verify our findings and adapt efficient health intervention.

[Sarcomatoid carcinoma of the kidney: a case report]. / Carcinome sarcomatoïde du rein: à propos d'un cas.

Sarcomatoid carcinoma of the kidney is an uncommon tumor associated with a very poor prognosis. Because this tumor can be occasionally difficult to distinguish from renal sarcoma, immunohistochemistry and electronmicroscopy are sometimes necessary for diagnosis. Therapy is actually essentially surgical because if adjuvant therapies should follow radical nephrectomy, no standardized regimen has been at present defined.

[Hepatic alveolar hydatidosis in Morocco]. / Hydatidose alvéolaire hépatique au Maroc.

This report describes a case of hepatic alveolar echinococcosis. To our knowledge it is the first documented report of human alveolar echinococcosis in Morocco. Alveolar echinococcosis of the liver is a relatively rare, severe chronic parasitic disease. It is characterized by slow-growing cysts that progressively invade and destroy the liver parenchyma like liver cancer. The discussion presents an update of the clinical, histologic, and therapeutic features of alveolar echinococcosis based on the findings in this case report and a review of the literature.

[Intra-abdominal desmoplastic small round cell tumor. A case report]. / Tumeur desmoplastique intra-abdominale à petites cellules rondes. A propos d'un cas.

Intra-abdominal desmoplastic small round cell tumor is an extremely rare and aggressive neoplasm that predominantly occurs in young adult men; it has an uncertain histogenesis, and predominant or exclusive intra-abdominal localisation without visceral origin involvement. This tumor is characterized by its distinct morphology and its multiphenotypic differentiation. We report a case of an intra-abdominal desmoplastic round cell tumor in a 24 years old woman. In the light of this case, the clinical morphological immunohistochemical and molecular of this rare desease are revewed.

[Gallbladder tuberculosis associated with cholelithiasis]. / La tuberculose de la vésicule biliaire: quel rôle de la lithiase?

Tuberculosis of the gallbladder is rare, even in our country known for being an endemic area. The positive diagnosis depends on suspicion of tuberculosis, peroperative findings and histological examination. From a review of the literature, the physiopathology of this infection is discussed, emphasizing the role of lithiasis in the development of tuberculous lesions. The authors report a case of gallbladder tuberculosis in a female patient who presented with a clinical picture of chronic cholelithiasis. The diagnosis of gallbladder tuberculosis was reached only after surgery and proven by histopathology. In our case, the presence of stones associated with non specific inflammatory alterations and possibly low resistance against tubercle bacillus, is believed to have been of importance for the development of the tuberculous infection.

[Pleurisy and sarcoidosis. Apropos of 2 cases]. / Pleurésie et sarcoïdose. A propos de 2 cas.

Pleural involvement is uncommon in sarcoidosis, observed in less than 5% of cases. We report two cases. Both patients were women. In the first patient, signs of sarcoidosis included pleural, mediastinal node, and multiple joint involvement as well as nasal obstruction, sicca syndrome and subcutaneous nodules. Diagnosis was established on pleural and nasal biopsies. For the second patient, in addition to the pleural involvement, there was interstitial lung disease and cervical, epitrochleal and deep abdominal node enlargement. Diagnosis was established from serial bronchial biopsies, pleural biopsies and peripheral node biopsies. Pleurisy is generally observed in cases with extensive lung involvement with extrathoracic localizations. Blind pleural biopsy is not very sensitive for diagnosis of sarcoidosic pleurisy. Light yellow exsudative fluid with a high lymphocyte count in a patient with a radiological and clinical presentation suggestive of tuberculosis, which must be ruled out, is a characteristic feature.

[Chordoma: atypical mediastinal mass. Report of a case]. / Le chordome: une masse médiastinale atypique. A propos d'un cas.

Chordoma is a rare tumor which develops from remnant notochord tissue. Sacro-coccygeal and spheno-occipital localizations predominate. We report a thoracic chordoma of the fifth thoracic vertebra with mediastinal expansion in a 70-year-old woman. Pathology confirmed the diagnosis revealing a lobulated architecture, presence of physaliphorous cells and intracellular mucoid substance. Immunohistochemistry can also be very helpful in atypical cases: positive for anti-cytokeratine, antivimetin and anti-protein S100 antibodies. Spontaneous outcome is fatal. Early and complete resection is the only way to improve prognosis.

[Fibrous tumor of the pleura]. / Tumeur fibreuse localisée de la plèvre.

Pleural fibroma, or fibrous tumor of the pleura, is an uncommon entity which is characterized by slow proliferation of undifferentiated, intermediary or mature fibroblasts associated with collagen fibers forming a tumor stroma. We report a case in a 49-Year-old man who developed exercise-induced dyspnea and right chest pain. The thoracic CT scan revealed the presence of a mass in the right lung base composed of heterogeneous encapsulated tIssue. Tumor resection was performed leading to the histological diagnosis of pleural fibroma. Immunohistochemistry tests revealed positive vimetin and CD34, and negative cytokeratin uptake. These immunohistochemistry data contributed to the differential diagnosis with malignant pleural mesothelium. Pleural fibroma is a benign tumor in 80% of the cases. Prognosis is excellent. Local recurrence is exceptional and generally occurs after incomplete resection. Radial surgical treatment determines the prognosis and is required to prevent local recurrence. Other criteria of malignancy are not correlated with the clinical course of this type of tumor.

Celiac disease, enteropathy-associated T-cell lymphoma, and primary sclerosing cholangitis in one patient: a very rare association and review of the literature.

Enteropathy-associated T-cell lymphoma (EATL) is a very rare peripheral T-cell lymphoma which is mostly associated with celiac disease. However, the association of primary sclerosing cholangitis and enteropathy-associated T-cell lymphoma is uncommon. Herein we report and discuss the first case of patient who presented simultaneously with these two rare diseases. It is a 54-year-old man who stopped gluten-free diet after 15 years history of celiac disease. The diagnosis was based on the histological examination of duodenal biopsy and the diagnosis of primary sclerosing cholangitis was made on liver biopsy, as well as the magnetic resonance cholangiogram. The treatment of EATL is mainly based on chemotherapy in addition to the optimal management of complications and adverse events that impact on the response to treatment and clinical outcomes, although the prognosis remains remarkably very poor.

[Intracranial malignant melanoma. Report of 4 cases]. / Les mélanomes malins intracrâniens. A propos de 4 cas.

We report 4 cases of intracranial melanoma without any clinically diagnosed extracerebral location. These tumors presented at different sites and had different histological aspects. Intracytoplasmic melanin was present in all cases, immunohistochemistry confirmed the diagnosis and eliminated a possible glioblastoma or metastatic carcinoma. Clinical and pathologic characteristics of primary pigmented lesions of the CNS are discussed with particular interest in diagnostic difficulties and histogenesis of these lesions.