Alveolar Soft Part Sarcoma Metastasizing to the Brain: A Rare Entity Revisited with Review of Recent Literature.

A 20-year-old male patient presented with a recent history of two episodes of seizures and Glasgow Coma Scale of E3V3M6. He was drowsy at presentation with bradycardia. On further examination, he was found to be having a 10 × 6cm well-circumscribed mass in the right medial thigh whose fine needle aspiration cytology was suggestive of soft tissue sarcoma. Contrast-enhanced magnetic resonance imaging brain showed enhancing lobulated lesions in bilateral hemispheres with significant edema. A decision to decompress the intracranial space occupying lesion was taken. Left-sided larger brain lesions underwent total gross excision followed by excision of the thigh mass. Histopathology of brain lesion was suggestive of metastatic deposits of alveolar soft part sarcoma, whereas the thigh mass was suggestive of alveolar soft part sarcoma grade 2 according to Fédération Nationale des Centres de Lutte Contre Le Cancer grading system. Postoperative course of the patient was unremarkable and his general condition improved remarkably (Functional Assessment of Cancer Therapy-Brain version 4 score improvement >50% at 1 month). He is in follow-up for the last 12 months and currently has completed chemoradiotherapy and is living a self-sufficient life. Though patients with aforementioned rare diagnosis have overall poor prognosis, decompressive craniotomy with metastasectomy may improve the overall quality of life of such young patients, and further chemoradiotherapy may help in prolonging life with acceptable level of comorbidity. This case is being discussed here in terms of the rarity of the final diagnosis, the management strategy used, and the quality of life improvement.

A solitary osteochondroma of the cervical spine: a case report and review of literature.

INTRODUCTION: Osteochondromas are usually found in the extremities and are rarely seen in the spine. They are most commonly found in the posterior elements of the spine, and intraspinal extension is uncommon. Compressive myelopathy as a presentation of vertebral osteochondroma in a child is a rare entity. METHODS: We report a case of vertebral osteochondroma arising from the lamina of C3 vertebra, presenting with features of compressive myelopathyin a 15 year old boy.Total excision of the tumor was carried out along with lamina of C3 vertebra.Patient recovered significantly. CONCLUSION: Spinal osteochondromas must be considered as rare etiology of spinal cord or root compression in the pediatric age group and utmost care should be taken while excising these benign lesions.

Traumatic elevated vertex fracture with delayed increase in intracranial pressure: a rare case.

INTRODUCTION: Skull fractures are traditionally classified into linear, comminuted or depressed which can either be simple or compound. A skull fracture where the bone fragment is elevated above the intact skull known as elevated skull fracture has been reported infrequently in literature. CASE PRESENTATION: We report a unique case of simple elevated vertex fracture in a 3-month-old child where the vertex had separated from the calvarium and was elevated above the level of outer table in a patient with delayed neurological deterioration. Cerebrospinal fluid leak into tight subgaleal space and gradual thrombosis of superior sagittal sinus could have led to late clinical deterioration. CONCLUSION: Prolonged monitoring, probably early repair of dural tear and aggressive management of raised ICP, is required. Reduction of fracture with careful manipulation of SSS should weigh the risk of exsanguination. No such case of an elevated vertex fracture has been reported so far in the literature.

Intracranial extradural hematoma: Spontaneous rapid decompression - not resolution.

The surgical option to evacuate an intracranial extradural hematoma (EDH) was postponed in a 2-year-old female child who appeared fully alert and active after a brief spell of unconsciousness following a fall from height. The child was received, with a swelling on and around the right parietal eminence, by the emergency staff just half an hour after the time of injury. The immediate X-ray skull and first computed tomography (CT) scan head showed a parietal bone fracture, EDH, and cephalhematoma. However, follow-up CT scan head after about 4½ h revealed the dramatic absence of EDH but increased size and bogginess of cephalhematoma. The EDH had transported into subgaleal space resulting in a decompression of intracranial compartment in <5 h, thereby preventing surgical intervention but necessitating monitoring, though there was no back flow intracranially.