Congenital isolated juvenile xanthogranuloma of the sole - a unique case report in a newborn.

Juvenile xanthogranuloma (JXG) is a rare, benign non-Langerhans cell histiocytosis that primarily affects the skin, with infrequent extracutaneous manifestations. Lesions typically emerge during early childhood and often resolve spontaneously, obviating the need for treatment. This paper details the case of a child diagnosed with a solitary JXG on the sole, necessitating surgical excision due to its functional impairment, specifically a delay in walking and weight bearing.

Expression of receptor for advanced glycation end-products (RAGE) in thymus from myasthenia patients.

OBJECTIVES: The receptor for advanced glycation end-products (RAGE) is a membranous immunoglobulin involved in the pathogenesis of numerous autoimmune diseases and tumors. The aim of this study was to investigate the possible involvement of RAGE in the pathogenesis of myasthenia gravis. MATERIAL AND METHODS: This prospective study included 41 cases of myasthenia gravis treated at our institution between 2010 and 2015. There were 18 men and 23 women, with an average age of 36.44±14.47 years. The majority of patients (24.4%) were classified as IIb, according to MGFA scoring, and 21 of them required corticosteroid and/or immunosuppressive treatment. Assessment of RAGE in thymus specimens was done by immunohistochemistry using RAGE antibody (C-term). RAGE expression was assessed according to various clinical, paraclinical and pathological parameters. RESULTS: Histopathological studies found 18 thymomas, 17 hyperplasias and six other types of pathology. Expression of RAGE was negative/weak in 19 cases and moderate/strong in 22 cases. It was more important in thymoma type B2 (P<0.001) and when the duration of myasthenia was short (P=0.04), and was not significantly related to either myasthenia clinical severity or preoperative treatment. CONCLUSION: Our results suggest that the RAGE pathway is involved in myasthenia gravis pathophysiology, especially at disease onset, and in forms with thymomas. Further studies would be indispensable to explore other aspects of this signaling pathway, especially the potential role of different ligands and soluble forms of RAGE.

[Retroperitoneal leiomyoma: a case report]. / Léiomyome rétro-péritonéal: à propos d'un cas.

Retroperitoneal leiomyoma is a rare benign tumor of the retroperitoneum. We report a clinical case of a 43-year-old patient, who suffered from back pain and weight loss. Imaging revealed a retroperitoneal mass, then the patient had a total excision of the tumor. Histological examination of the surgical specimen concluded to retroperitoneal leiomyoma. The evolution was good without recurrence after 12 months.

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.

Pathological subtrochanteric fracture revealing a primary hyperparathyroidism: A case report.

INTRODUCTION: Primary hyperparathyroidism is the third most common endocrine disorder after diabetes and thyroid disease. Nevertheless, a pathological fracture revealing primary hyperparathyroidism is not commonly described. CASE PRESENTATION: We present the case of a 30-year-old male patient who was admitted with a subtrochanteric pathologic fracture of her left femur after a trivial fall. Due to suspicion of bone metastases, a thoraco-abdomino-pelvic CT scan was requested. It revealed multiple irregular circumscribed lytic bone lesions in the ribs, the right scapula, the dorsal spine, and the pelvic girdle; bilateral renal lithiasis; and a nodule contiguous to the left posterior thyroid lobe. A surgical biopsy of the bone lesion was performed. Histopathological examination confirmed the diagnosis of primary hyperparathyroidism with a brown tumor. Laboratory tests showed increased both calcium and PTH levels. The patient underwent cephalomedullary nail fixation with bipolar locking. After that, a parathyroidectomy was performed. At six months' follow-up, the functional result was satisfactory. CLINICAL DISCUSSION: Primary hyperparathyroidism is the most common cause of hypercalcemia. It occurs as a result of a parathyroid adenoma in 80 % of cases. Definitive diagnosis should be made by clinical history, radiological findings and confirmed by biochemical tests including serum parathyroid hormone (PTH), alkaline phosphatase, calcium, phosphate, and vitamin D levels. CONCLUSION: This case report emphasizes the need of including brown tumors in the differential diagnosis of multifocal osteolytic bone lesions, in order to ensure appropriate treatment.

[Duodenal angiodysplasia: an unusual cause of intestinal obstruction]. / Angiodysplasie duodénale: cause inhabituelle d'occlusion intestinale.

Angiodysplasia is a relatively rare lesion that however may be a major source of upper and lower gastrointestinal bleeding. It's mostly related to the aging and degeneration of the blood vessels, as it occurs in older adults. Clinical presentation is variable, ranging from asymptomatic cases over iron deficiency anaemia to acute or recurrent bleeding. We present a case of 60 year-old woman presented with anaemia and intestinal obstruction. Diagnosis can usually be made using endoscopy, sometimes with additional biopsy. Treatment can be symptomatic, including iron supplements and transfusion therapy or causal, including therapeutic endoscopy (laser, electrocautery, heater probe or injection sclerotherapy), therapeutic angiography and surgery.

[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.

[Hydatidosis: apropos of several uncommon locations]. / Hydatidose: à propos de quelques localisations inhabituelles.

Most hydatid cysts are found in the liver or lungs but occurrence in other locations is possible. The purpose of this report is to describe three cases involving cysts locate in the ovary, joint, and thyroid. Clinical findings associated with these uncommon locations were poorly specific and diagnosis required histological examination after surgical exploration. Imaging and serology were useful to differentiate hydatid cyst from benign or malignant tumor disease but often failed to achieve definitive differential diagnosis. Thorough investigation is necessary to rule out more common locations in the liver or lung. Continued surveillance is needed for early detection of recurrence. Preventive campaigns are necessary in endemic areas, such as Morocco.

[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.

[Pulmonary metastasis from a glioblastoma. A case report]. / Métastase pulmonaire d'un glioblastome. A propos d'un cas.

Glioblastoma is a primary malignant astrocyte tumor of the central nervous system. Extraneural metastasis is uncommon. We report a case of spontaneous lung metastasis from a glioblastoma without prior surgery. Positive diagnosis was achieved from histology and immunohistochemistry

[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.

[Adamantinoma of the clavicle: rare tumor for rare location]. / Localisation inhabituelle d'une tumeur osseuse rare : l'adamantinome de la clavicule.

Adamantinoma is a rare primary low-grade malignant tumor composed of cells with epithelial and fibrous characteristics. It represents 0.4% of all primitive malignant bone tumours. It is predominantly located in the mid-shaft of tibia. We report an adamantinoma of the clavicle, occurring in a 19-year-old female patient. The lateral half of the clavicle was excised. After a period of 3 years, she is still remaining free of local recurrence and metastatic disease.

[Inclusion body fibromatosis: a case report]. / Fibromatose digitale infantile : à propos d'un cas.

Infantile digital fibromatosis or inclusion body fibromatosis is a rare, benign fibroproliferative lesion with recurrent potential that occurs on the digits of infants. A highly characteristic morphologic finding is the presence of paranuclear inclusion within the tumoral cells. We report here a case occurring in an 8-month-old infant with 2 asynchronous lesions of the toes.