ABSTRACT
Thalassemia is known to induce extramedullary hematopoiesis (EMH), which is a compensatory mechanism in which the body forms blood cells outside the bone marrow. While EMH typically affects organs such as the spleen and liver, there are rare instances where it leads to spinal cord compression (SCC) in the epidural space. A 31-year-old male patient with transfusion-dependent beta thalassemia presented with numbness and bilateral limb weakness due to EMH. Neurological examination revealed increased tone in both legs, reduced power, loss of crude touch and pain sensation, and increased deep tendon reflexes. Magnetic resonance imaging (MRI) indicated a lobulated soft tissue structure in the posterior dural intrathecal space causing SCC. Laminectomy of the T2-T8 vertebrae was done, after which the lesion was identified and completely removed. Post-surgery, significant neurological improvements were observed in both motor and sensory functions. Thalassemia patients presenting with symptoms of SCC should be investigated for the presence of epidural EMH. Treatment options include decompressive surgery, blood transfusions, hydroxyurea, and radiotherapy.
ABSTRACT
Giant cell tumors (GCTs), typically benign, predominantly manifest in individuals aged 20-40, with the most common locations being the metaphysis or epiphysis of the femur or tibia. Infrequently, they may occur in the skull. Despite their benign nature, these tumors can exhibit aggressive behavior and have the potential to metastasize. In the case at hand, a 20-year-old female presented to the hospital with a progressively enlarging right frontal swelling over the preceding months. The patient reported intermittent headaches, alleviated by analgesics, and exhibited a normal neurological examination along with a Glasgow Coma Scale (GCS) score of 15 out of 15. Imaging revealed an expansive soft tissue mass in the right frontal bone involving both inner and outer tables. Surgical intervention was pursued through a right frontal incision followed by tumor excision. Histopathological examination of the specimen confirmed the presence of a GCT. The limited existing literature on this topic highlights the need for further research and insights into effective strategies. This case contributes to addressing this gap in knowledge, offering valuable information to enhance our understanding of the challenges associated with similar rare cases and improve patient outcomes.
ABSTRACT
OBJECTIVES: To investigate the clinical and histopathological characteristics, with the prognostic factors, treatment outcome, pattern of relapse, and survival analysis of uterine sarcoma patients. METHODS: All patients with histologically proven uterine sarcoma were identified using the database at King Abdulaziz University Hospital, Jeddah, Saudi Arabia between January 2000 and December 2012. RESULTS: A total of 36 patients with uterine sarcoma were reviewed. The median age of all patients was 57 years, and the mean age was 57.72+/-13.17 years. Carcinosarcoma was reported in 21 patients (58%), leiomyosarcoma in 7 (19%), undifferentiated endometrial sarcoma in 6 (17%), and rhabdomyosarcoma in 2 (6%). Approximately half of the patients were stages III and IV (28% and 25%), while 15 patients (41%) were stage I; only 2 patients (6%) were stage II. The surgical treatment was hysterectomy and bilateral salpingoophorectomy (H+BSO) plus staging in 18 patients (50%), while in 4 patients (19%), H+BSO plus debulking was performed. Adjuvant chemotherapy was given in 24 (69%) and adjuvant radiotherapy in 5 (14%) cases, At a median follow-up period of 13.5 months, 8 patients (22%) relapsed. The 2-year disease-free survival (DFS) rate was 22% and the 5-year was 14%. In the multivariate analysis, the advanced stages (p=0.015) and lymph vascular invasion (p=0.0001) were associated with poor DFS, while the use of chemotherapy significantly improved the DFS (p=0.027). CONCLUSIONS: The poor outcome of high-grade uterine sarcoma patients was identified, and only one third of patients (30%) survived for 2 years. This finding necessitates the need for more aggressive tools to fight this disease.
