Anesthestic management of Kassabach-Meritt Phenomenon in an adult: case report.

BACKGROUND: Kasabach-Merritt phenomenon (KMP) is characterized by a vascular tumor with profound thrombocytopenia and consumptive coagulopathy that may presents significant challenges for anesthesiologist. CASE PRESENTATION: An 87-year-old man presented with kaposiform hemangioendothelioma involving the right leg in critical condition due to massive bleeding. Hematology investigations indicated the presence of KMP. Association of this type of tumor with KMP in adults has never been reported. CONCLUSION: The present case report lays an emphasis on the potential difficulties during anesthetic management of this rare condition.

Late Diagnosis of Primary Hyperoxaluria in an Adult Patient With End-Stage Renal Disease and Bicytopenia.

Primary hyperoxaluria (PH) is a rare genetic condition that disrupts the normal process of glyoxylate metabolism, resulting in an overproduction of oxalate. This excessive oxalate production leads to the accumulation of calcium oxalate (known as oxalosis) throughout various organs in the body. The urinary tract, specifically the renal parenchyma, is the first location where the deposition of calcium oxalate begins in PH. These deposits are responsible for nephrocalcinosis and tubule­interstitial nephritis which leads to end­stage renal failure. This is then followed by the accumulation of oxalate in other organs including the bone marrow. Herein, we report the case of a 22-year-old male patient who presented with bicytopenia; he had a history of end-stage renal disease preceded by recurrent urolithiasis and nephrolithiasis episodes since the age of 3 years. A bone marrow biopsy was performed for evaluation of the bicytopenia which led to the diagnosis of PH.

Tuberculosis of the Tibial Plateau Mimicking a Giant Cell Tumor: A Case Report.

Tuberculous osteomyelitis is infrequent and occurs most often in the femur, the tibia, and the small bonne of hands and feet. Herein, we report a 39-year-old female who presented with chronic pain and motion range reduction of the left knee joint for two years. A knee radiograph revealed a geographic lytic lesion of the epiphyseal and diaphyseal region of the tibia mimicking giant cell tumor (GCT). A minimally invasive biopsy of the lytic lesion was performed, and pathological assessment revealed granulomatous inflammation made of numerous caseating necrotizing epithelioid and giant cells granulomas, diagnostic of tibial plateau tuberculosis. This case underscores the importance of taking tuberculosis into consideration in lesions mimicking GCTs in the tibial plateau, especially in endemic regions.

Disseminated peritoneal leiomyomatosis: A case report.

Disseminated peritoneal leiomyomatosis (DPL) or leiomyomatosis peritonealis disseminata is a sporadic benign disease characterized by several solid peritoneal smooth muscle nodules that proliferate along the abdominopelvic cavity. The source of the condition is undetermined, although suspected causes include iatrogenic and hormonal stimulation. It primarily affects women of reproductive age. Imaging investigations are important in determining the extent of lesions and the presence of malignancy. There are no conventional therapeutic guidelines for the therapy of DPL, hence the risk of malignant transformation is low. We discuss the case of a 41-year-old woman who had a previous laparoscopic hysterectomy and presented 4 years later with numerous peritoneal tumors the diagnosis of DPL was suspected by computed tomography and magnetic resonance imaging, and confirmed by histology.

Saphenous Nerve Schwannoma: A Rare Differential Diagnosis of Knee Pain in Children.

Schwannomas are uncommon benign tumors of the peripheral nerves with a low risk of malignant transformation. They rarely affect children, can affect any part of the body but rarely occur in the lower extremity and typically present with a palpable mass, pain or neurological signs. Imaging helps to orient the diagnosis and anatomopathological examination helps to confirm it. We report a case of a 12-year-old girl who presented with left knee pain with subcutaneous mass overlying the tibial tuberosity medially. Clinical examination revealed a positive Tinel's sign. Magnetic resonance imaging (MRI) of the knee was performed, which revealed an encapsulated subcutaneous soft tissue mass overlying the tibial tuberosity medially, eccentric to the course of the infrapatellar branch of the saphenous nerve. The patient was operated with total intracapsular excision of the lesion and the anatomopathological study of the surgical specimen came back in favor of a schwannoma. Postoperatively, the patient showed a good recovery with disappearance of pain and swelling.

A rare case of Ewing's sarcoma of the maxillary sinus.

Ewing's sarcoma is generally an aggressive, poorly differentiated bone and soft tissue tumor affecting children and young adults, it accounts for 4%-6% of all primary bone tumors and primary facial locations occur in only 1%-4% of all cases, primarily in the mandible and calvaria. Involvement of the paranasal sinuses is rare. Here we report the case of an 11-year-old girl with no medical, surgical, or traumatic history, who presented for 1 month and progressive evolution of swelling of the left cheek, associated with pain, nasal obstruction, rhinorrhea, and a slight weight loss not quantified. A craniofacial computed tomography (CT) scan showed a mixed lytic and condensing lesional tissue process centered on the left maxillary sinus, heterogeneously enhanced after contrast injection, lysing the walls of the sinus extended to the homolateral nasal cavity and slightly infiltrating the adjacent soft tissues. An incisional biopsy was performed and the pathological study proved that it was Ewing's sarcoma. She was put on neoadjuvant chemotherapy using 6 courses of vincristine, doxorubicin, ifosfamide, etoposide which resulted in a partial regression of the tumor size by 50%. Then the patient was put on combined chemotherapy and radiotherapy. A follow-up CT scan after 6 courses of vincristine, actinomycin, cyclophosphamide, and 17 sessions of radiotherapy showed lesion stability. Maxillary Ewing's sarcoma is a rare and aggressive tumor. Therefore, early diagnosis, combination therapy, and long-term follow-up are suggested in such cases to improve the survival rate.

Unusual Etiology of Chronic Posterior Leg Pain in a Running Athlete: Could It Be a Schwannoma? A Case Report and Review of the Literature.

Schwannomas represent only 5% of all soft tissue tumors. As a variant of this tumor, the plexiform schwannoma is rare accounting for less than 5% of all schwannomas. Herein, we report a rare case of a 49-year-old athlete who suffered from a pain in the posterior aspect of the right leg one year before his presentation. Initially, a radiograph of his right leg showed no abnormality, and so, the emergency physician discharged him on analgesics and anti-inflammatory medications, and rest was advised. The persistent pain obliged the patient to consult our orthopedic department. On examination, we found a firm mass in the proximal medial aspect of his right leg. The neurovascular exam was normal. Sonography of the leg was not conclusive. Therefore, magnetic resonance imaging was performed, and a hemangioma or schwannoma was suspected. The patient underwent surgery in which the entire tumor mass was shelled out in one piece with no damage. The histopathological finding was concomitant with a plexiform schwannoma. Follow-up evaluation, sixteen months later, showed no evidence of recurrence, and the patient has regained his previous level of sportive activities. So, given the case described here, despite the rarity of the schwannoma, it should be taken into consideration as a possible diagnosis in such situation to promote early diagnosis and appropriate treatment.

Lymphoepithelioma-Like Carcinoma of the Bladder: A Case Report of a Rare and Particular Variant of Urothelial Carcinoma.

Lymphoepithelioma-like carcinoma of the bladder (LELCB) is a rare variant of urothelial carcinoma first described by Zukerberg in 1991 and confirmed as a variant of urothelial carcinoma by the WHO classification of tumors of the urinary system. LELCB is characterized by a marked infiltration of lymphocytes in the area involved by the tumor which may coexist with the conventional urothelial carcinoma. LELCB are classified according to the percentage of lymphoepithelioma component within the tumor with the prognosis depending on the percentage. We report a new case of pure LELCB occurring in 63-year-old woman presenting with hematuria. Ultrasonography and cystoscopy revealed a large tumor on the left lateral wall of the bladder. Transurethral resection of the bladder tumor (TURBT) was performed. Pathological and immunohistochemical analysis revealed a high-grade muscle-invasive LELCB (G3pT2). The patient underwent an adjuvant systemic chemotherapy with no recurrence after a ten-month follow-up. To our knowledge, this is the second Moroccan case of LELCB reported in the English literature. Although its rare occurrence prognosis and ideal therapeutic management of LELCB have not been clearly established yet, literature findings encourage the adoption of a conservative approach in treatment of LELCB.

[Uncommon mammary-type myofibroblastoma unlike any other: about a case]. / Un myofibroblastome de type mammaire pas comme les autres: à propos d'un cas.

Mammary-type myofibroblastoma (MTMF) is a rare benign mesenchymal tumor, initially detected in the breast. Its diagnosis is based on a spectrum of clinical, radiological and possibly histological parameters. We here report the rare case of a 50 year old male patient with mammary-type myofibroblastoma located in the axillary region. The peculiarity of our case lies in the presence of numerous mitoses and this was rarely described in the literature. The aim of our case study was to raise the problem of differential diagnosis of this rare tumor.