Massive lingual teratoma in a neonate.

Teratoma of the tongue is a rare entity. We present a male newborn with massive lingual teratoma and cleft palate, which surprisingly did not cause immediate airway obstruction. This case illustrates a huge mass in the oral cavity, which was missed on antenatal ultrasonography because it did not present with polyhydramnios. The mass was excised under general anaesthesia. Histopathologically, it consisted of all three layers of embryonic elements with predominantly glial tissue. Postoperatively, the patient developed hypoglossal nerve palsy, and no recurrence was detected after four years.

Arteriovenous malformation of the pregnant uterus.

Arteriovenous malformation of the pregnant uterus is very rare, and may present with unexplained torrential bleeding. We report a patient with absence of the conventional risk factors, and was saved by quick recourse to hysterectomy to control the bleeding.

Third ventricular cavernous angioma.

Third ventricular cavernous angiomas are rare vascular malformations of the brain. We report an eight-year old boy with a rare third ventricular cavernous angioma that hemorrhaged presenting with symptoms of acute hydrocephalus. Computed Tomography (CT) and Magnetic Resonance Imaging (MRI) showed a heterogenous ill-defined, solid and cystic intraventricular mass in the third ventricle which was mildly enhanced with contrast and there was associated hydrocephalus. The mass was removed with success and follow up after two years revealed no neurological abnormalities.

Aggressive giant cell tumour of bone.

INTRODUCTION: The surgical treatment of Stage III or aggressive giant cell tumour of the bone, whether to perform intralesional or en-bloc resection, remains controversial. The aim of this study is to identify the effectiveness of en-bloc resection for local control and final oncological outcome of the disease. METHODS: The data of 20 consecutive patients with Stage III giant cell tumour were retrospectively reviewed to determine the local control and oncological outcome after treatment with wide resection. RESULTS: The majority of the patients presented late with mean duration of symptoms of 24 months, and four patients presented with recurrences. All patients were treated with wide resection except for two patients who underwent ablative surgery due to major neurovascular involvement. Ten patients required free vascularised tissue transfer to cover massive soft tissue defect. Local recurrence occurred in one patient who was again treated with wide resection and vascularised flap. Six patients had pulmonary metastases. Two patients with resectable disease were treated with thoracoscopic surgery and they remained disease-free 36 months after surgery. Two patients with multiple lung metastases were treated with chemotherapy and the disease remained non-progressive. The remaining two patients who refused chemotherapy showed radiological progression, and one succumbed to the disease with massive haemoptysis. CONCLUSION: Aggressive giant cell tumour of bone should be treated with wide resection for better local control, and treatment of pulmonary metastases is mandatory for overall prognosis.

Osteosarcoma: the outcome of limb salvage surgery.

We reviewed the surgical and oncological management 23 consecutive patients with osteosarcoma of the long bones to determine the outcome of limb salvage technique performed in our centre. All patients received neoadjuvant chemotherapy. There were 15 males and 8 females with a mean age at diagnosis of 19 years (9 to 36). The median follow-up was 30 months (10 to 60). Fifteen had lesion around the knee joint followed by three in the proximal humerus, two in distal humerus, two in the pelvis, and one in the distal tibia. Six patients presented with lung metastases at diagnosis. We performed limb salvage surgery to control local disease in 16 patients and amputation in 7. The resection margins of the primary lesion were adequate and free of tumour cells in all patients. Local recurrence developed in 1 patient of limb salvage group. The overall median survival was 22 months and actuarial survival was 52% at 3 years. Eleven patients died of pulmonary metastases within 2 years of follow-up. Median survival of the limb salvage surgery group was 30 months compared to 6 months in the amputation group. As per our experience, limb salvage technique is a feasible option in extremity osteosarcoma without compromising survival.

Natural history of giant cell tumour of the bone.

The clinical presentation and behaviour of giant cell tumours of bone vary. The progression of the disease and metastases are unpredictable, but the overall prognosis is good. We describe the natural history and different clinical presentations of two cases of giant cell tumour of bone where the patients had refused the initial treatment and presented several years later with the disease.

Hepatoblastoma in a low birth weight infant : a case report and review of the literature.

Hepatoblastoma is the most common primary liver tumour of childhood. This is a case report of a one-year-old boy who presented with a one-month history of progressive abdominal distension and weight loss. He was cachexic, anaemic, had gross hepatomegaly and ascites. He had been born prematurely with a birth weight of 1.23 kg, and his developmental milestones were delayed. Ultrasound and CT scan demonstrated a large solid tumour in the left lobe of the liver with a smaller superficial nodule in the right lobe. Serum alpha fetoprotein was significantly raised. A left lobe hepatectomy and complete excision of the right sided nodule was performed. There was no evidence of metastatic disease. Histopathological examination confirmed hepatoblastoma of the fetal type. The patient developed features of intestinal obstruction a few days after surgery and he succumbed ten days after re-laparotomy. The clinical presentation and investigation results in this case are characteristic. Recent reports have suggested a strong relationship between very low birth weight (< 1500gm)/prematurity and hepatoblastoma as is present in this case. Surgery is the mainstay of therapy in hepatoblastoma. A brief review of the literature on this tumour is presented.

Anterior uveal and episcleral metastases from carcinoma of the breast.

Breast carcinoma is the most common primary tumor producing intraocular metastasis. Metastases to the iris and ciliary body are relatively rare. The authors report a case of a 61-year-old lady, operated for carcinoma of the left breast 3 years back, who presented with symptoms and signs of acute narrow-angle glaucoma in the right eye. A diffuse whitish plaque-like mass in the upper nasal quadrant of the iris with an episcleral nodule on the limbus in the corresponding area and all the signs of acute narrow-angle glaucoma were present in the right eye. Intraocular pressure was controlled medically. Fine-needle aspiration cytology from the episcleral nodule showed malignant cells. Histopathology of the excised nodule showed metastatic poorly differentiated carcinoma, and the cellular pattern was similar to the carcinoma of the breast. There was no other metastasis anywhere in the body. Fine-needle aspiration cytology from an external lesion of the eye is a less invasive and easier procedure than paracentesis to diagnose the metastatic nature of the lesions. The rare features in our case are the clinical presentation as acute glaucoma and the ocular structures being the first and only site of metastasis.

Fisher's unique variant in Stocker's type II congenital cystic adenomatoid malformation of the lung: a case report.

An eight-week-old infant presented with dyspnoea two months after an uneventful normal vaginal delivery. Radiologically, a sharply outlined radiolucent area surrounded by atelectasis was seen in the upper lobe of the left lung. A left upper lobectomy was performed with the clinical impression of congenital pulmonary emphysema. The resected specimen displayed multiple cysts 2 to 6 mm in diameter. Microscopically, intracystic papillary mesenchymal ingrowths lined by respiratory epithelium were present. Based on both the gross and microscopical features, a diagnosis of Fisher's variant of type II congenital cystic adenomatoid malformation (CAM) was made. The postoperative follow-up showed excellent recovery and normal development of the child.

Pulmonary paragonimiasis: clinical features, diagnosis and treatment of 39 cases in Manipur.

Thirty-nine cases of pulmonary paragonimiasis due to Paragonimus westermani were identified in Manipur, India. Recurrent haemoptysis, often increased on exertion, was the most common symptom. The clinical features and radiological appearances simulated pulmonary tuberculosis and 23 cases were treated for tuberculosis for varying periods before the final diagnosis. Young males aged 11 to 30 years comprised two-thirds of the cases. The disease was acquired by eating raw or improperly cooked crabs (Potamon deham). Intradermal tests (IDT) employing a saline extract of adult Paragonimus were a simple and reliable method of detecting present or past infection. Erythema of large size even without wheal and pseudopodia may indicate a positive IDT. Charcot-Leyden crystals (CLC) were detected in all sputum samples positive for Paragonimus ova. In patients (four) presenting with pleural effusion, Paragonimus ova were not present in the sputum, stool or pleural fluid. Bithionol (2,2'-thiobis [4,6-dichlorophenol]) orally, 40 mg/kg body-weight/day, 10 to 25 doses, cured all cases and, except in one, side effects were minimal. Relapse in one patient after six months' treatment was apparently cured by a second course of Bithionol.

Pathological aspects of diffuse nodular hyperplasia of the liver.

The microscopical features in specimens of liver tissue from 14 patients considered to be suffering from Diffuse Nodular Hyperplasia (DNH) were compared with the changes found in livers from five other groups of patients. The diagnoses in these groups were macronodular cirrhosis, hepatic venous occlusion, congestive cardiac failure, compensatory hyperplasia after destruction of part of the liver and chronic biliary disease respectively. In all these groups the formation of thick hepatocyte plates, thought to represent cell proliferation, was a constant and striking feature. Such plate thickening in the periportal regions was associated with congestive cardiac failure and with hepatic venous occlusion. The diffuse involvement of almost all plates formed part of the changes in DNH, cirrhosis, compensatory hyperplasia and chronic biliary disease. Normal vascular relations were retained in the patients with compensatory hyperplasia and chronic biliary disease and lost in cirrhosis and, in some parts, in DNH. The nodules were small in DNH and larger in cirrhosis, where they were surrounded by fibrous tissue. DNH appeared to be recognizable in needle biopsy specimens.

Intrahepatic biliary papillomatosis.

Intrahepatic bile duct papillomatosis associated with changes of nuclear atypia is described in a young man. Radical excision was performed by left hepatic lobectomy and removal of the common bile duct and left hepatic ducts. Fourteen previous cases of intrahepatic or diffuse intrahepatic and extrahepatic biliary papillomatosis are reviewed. While local recurrence and progression to death from obstructive jaundice and cholangitis is the rule, only two definite cases of malignant change have been reported. Radical surgery may be curative for this rare condition.

Pathological aspects of cholangiocarcinoma.

Tissue specimens resected from liver and bile-ducts were examined in an attempt to distinguish features of cholangiocarcinoma from those of other tumours and from reactions to duct obstruction and changes in the tumour-bearing liver. Macroscopical and microscopical features were compared and certain changes were found only in cases of cholangiocarcinoma. The type of tumour correlated moderately well with the level of bile duct involved and some microscopical findings were noted only in patients suffering from cholangiocarcinoma. Mucin was demonstrable in all 33 cholangiocarcinomas. Other striking features included heterogeneity of epithelial cells within the same acinus and a tendency to spread between hepatocyte plates, along duct walls and in relation to nerves. Reactive hyperplasia of ductal subepithelial mucous glands was found in association with duct obstruction with and without cholangiocarcinoma. Sclerosis, a common accompaniment of cholangiocarcinoma was noted in other tumours.