Evaluation of Multimodality Management of Adenoid Cystic Carcinoma of the Head and Neck.

Adenoid cystic carcinoma is a relatively rare tumour arising from salivary glands of head and neck region. Surgery and radiotherapy form standard treatment modalities in the management of this tumor. In this analysis we present results of multimodality treatment in our cohort of patient. This retrospective analysis evaluated results of treatment in forty patients diagnosed with adenoid cystic carcinoma of the head and neck. Evaluation was done to identify prognostic factors affecting the disease free survival. A median disease free survival of 34 ± 2.42 (Median ± SE) versus 10 ± 5.45 months was seen in patients undergoing surgery followed by post operative radiotherapy versus radiotherapy alone (P = 0.01). A radiotherapy dose more than 60 Gy was associated with a better disease free survival compared with patients receiving less than 60 Gy (P = 0.01). Positive surgical margins and perineural invasion were associated with a poor treatment outcome (P = 0.02) Patients with c-kit positive status showed a poor local control rate (P = 0.05).

Hemorrhage in astroblastoma: An unusual manifestation of an extremely rare entity.

Astroblastoma is a rare tumor of glial origin with characteristics of both astrocytoma and ependymoma. It is usually seen in children and young adults, and is peripherally located, well circumscribed, of solid-cystic composition and with heterogeneous contrast enhancement. Histopathology reveals perivascular pseudorosette formation and thick hyalinised vessels. Hemorrhage in astroblastoma is unusual and rarely described in literature. We report two patients with astroblastoma who presented with hemorrhage and discuss the natural history, radiological findings, pathophysiology of hemorrhage and histopathological characteristics. We emphasize the importance of early suspicion in peripherally located lesions with bleeding.

Intraosseous schwannoma of the frontal bone.

Schwannomas or neurilemomas are relatively frequent benign tumors; however, intraosseous schwannomas are rare. The most common site for intraosseous schwannoma is the mandible. Other sites include the vertebra, ulna, humerus, femur, tibia, patella, scapula, rib, and small bones of the hands. The skull vault is a rare site for intraosseous schwannoma, and only 4 cases have been reported previously in the literature. This article presents a case of intraosseous schwannoma in the frontal bone in an 11-year-old boy. Radiologically, intraosseous schwannomas are lytic, sharply demarcated defects that often expand the involved bone. Perilesional sclerosis may surround the area of central lucency. Histologic features of intraosseous schwannomas are similar to that observed for soft-tissue schwannomas including Antoni type A areas (compactly arranged spindle-shaped cells with palisading or Verocay bodies) and Antoni type B areas (widely separated cells with loose intervening collagenous matrix). In our patient, typical radiologic and histologic findings including immunohistochemistry and electron microscopy results led to a diagnosis of intraosseous schwannoma.

Amebic meningoencephalitis: spectrum of imaging findings.

Primary amebic meningoencephalitis and granulomatous amebic meningoencephalitis are central nervous system infections caused by free-living amebae. We describe the neuroimaging findings in 5 such cases on CT and MR imaging. A spectrum of findings was seen in the form of multifocal parenchymal lesions, pseudotumoral lesions, meningeal exudates, hemorrhagic infarcts, and necrosis in the brain. Familiarity with the imaging findings is important for the diagnosis and management of this nearly universally fatal disease.

Vascular transformation of sinuses in lymph nodes associated with myelodysplastic syndrome--a case report.

We describe a case of plexiform variant of vascular transformation of lymph nodes sinuses in association with myelodysplastic syndrome. The patient had repeated bacterial infections and terminal fungal infection and dies after a protracted illness of seven years.

Extended survival of carcinoma head of pancreas following palliative treatment.

A 48 year old woman presented with obstructive jaundice 10 years back. Upper gastrointestinal endoscopy revealed a growth infiltrating the ampulla of Vater, which was confirmed to be adenocarcinoma on cytology. At laparotomy, a large nodular growth was seen in the head of pancreas. Surgical resection could not be done because of encasement of superior mesenteric vessels, hence a cholecystojejunostomy was performed. The patient remained asymptomatic for 9 years, when she developed cholangitis. Duodenoscopy at this stage revealed an ulcerated growth at the ampulla and biopsy from the growth confirmed a well differentiated adenocarcinoma. A straight flap 10 F stent was placed in the common bile duct. Thereafter the patient has remained asymptomatic for more than a year.

Congenital hepatic fibrosis in Indian children.

BACKGROUND: Congenital hepatic fibrosis (CHF) is an uncommon cause of portal hypertension in children. So far, there is no report of this from the subcontinent. We have studied the clinical spectrum of CHF in North Indian children. METHODS: Fifteen children were diagnosed with CHF on the basis of their liver histology over a period of 6.5 years. Their clinical details were recorded. Oesophagogastroduodenoscopy and abdominal ultrasonography were performed in all cases. All siblings were examined clinically; and ultrasonography, endoscopy and liver biopsy were performed if there was firm hepatomegaly. Children with variceal bleeding were managed by endoscopic sclerotherapy. The median age of these children was 8 years with a male to female ratio of 1.5:1. RESULTS: Only one sibling (of 33) was diagnosed as having CHF. The predominant presentations were variceal bleeding in six, abdominal distension in seven and incidental detection of organomegaly in two. Hepatomegaly was present in all patients and splenomegaly in all but one. Liver function and renal function tests were normal in all children, except for a raised serum alkaline phosphatase in six. Two children had associated renal cysts, two had choledochal cysts, one each had Caroli's disease and biliary atresia and two children had portal vein thrombosis. Variceal obliteration was achieved in five children after an average 4.8 sclerotherapy sessions and one required a mesocaval shunt. On follow up (median 41 months, range 1-80 months) all are doing well. CONCLUSIONS: Congenital hepatic fibrosis is mainly sporadic in India and associated renal lesions are uncommon. Endoscopic sclerotherapy is effective in controlling variceal bleed and the prognosis is universally good in the absence of renal diseases.