Vanishing bone disease (Gorham's disease) - A rare occurrence of unknown etiology.

A 20-year-old male patient presented with painful swelling around left elbow joint. Radiographic examination revealed osteolytic lesion with pathological fracture of lower end of humerus and upper radius. Upper end of ulna was completely absent along with bony erosion. Histopathology of the bony tissue revealed hemangioma-like lesion composed of vascular channels lined by benign endothelium replacing bone. The diagnosis of Gorham's massive osteolysis was made. Gorham's disease is a benign self-limiting condition affecting any age, may involve any part of the skeleton and is characterized by replacement of bone by hemangiomatous tissue resulting in formation of lesions exhibiting massive osteolysis, which may be to the extent of disappearance of the affected bone in radiograph. This nonhereditary case was not associated with nephropathy, which is often a coexistent condition. The case is being reported for its rarity.

A clinicopathological study of malignant melanoma with special reference to atypical presentation.

Malignant melanoma is a tumor of melanocytic origin. Lymphatic and hematogenous metastases are common in this condition. Retrospective analysis was performed in 16 consecutive cases diagnosed histopathologically as malignant melanoma at the pathology department of a medial college in eastern India. 75% of the patients were male; majority of them was in their sixth decade. All (100%) the lesions were pigmented. The primary site was known in all cases, except two (12.5%). Out of the 14 cases with known primary site 11 (78.57%) were cutaneous melanomas, including one arising in labia minora, two (14.29%) were ocular and one (7.14%) was vaginal in origin. Among cutaneous melanomas, superficial spreading type was the commonest variety and mixed population of epithelioid and spindle cell was the commonest histopathological pattern. The commonest grade of invasion was grade III (Clark's). The clinical presentation of the case of vaginal melanoma and the two cases of secondary melanomas, including the one with obscure primary tumor, were bewildering and hence are discussed separately.

A cross-sectional study on aqueous humour lactate dehydrogenase level in retinoblastoma.

Lactate dehydrogenase enzyme is a non-specific marker found raised in different body fluids in various pathological conditions. This cross-sectional study was done in a medical college of Kolkata, India to find out lactate dehydrogenase level of aqueous humour in retinoblastoma cases. Aqueous humour lactate dehydrogenase level from 21 histology proved retinoblastoma cases and 21 controls was estimated and compared. Range of lactate dehydrogenase levels in retinoblastoma cases were 863-4819 IU/l; mean being 3396.9 IU/l; and in controls range was 48-146 IU/l; mean being 90.6 IU/l; 76.19% of retinoblastoma cases had value over 3000 IU/l. None of the controls had value above 150 IU/l. The difference between lactate dehydrogenase values of cases and controls was significant (p<0.001).

Posthepatitis aplastic anaemia presenting only with bilateral vision loss.

Aplastic anaemia is pancytopenia with marrow hypocellularity. Hepatitis-associated aplastic anaemia is a varient or aplastic anaemia that follows an acute attack of seronegative hepatitis. Here a case of hepatitis-associated aplastic anaemia presenting with sudden onset of severe simultaneous bilateral vision loss and without any other usual presenting signs is reported. Partial recovery of blood cell count occurred following immunosuppressive therapy. Posterior hyalodotmy helped rapid resolution of premacular subhyaloid haemorrhage. Although bilateral vision loss may rarely be the initial presentation of aplastic anaemia, no such report is known in hepatitis-associated aplastic anaemia. Posthepatitis vision loss needs careful investigation to exclude an underlying haematological disorder.

An observational study on retinoblastoma cases attending a medical college in Calcutta.

Excision of the eyeball was done in 23 cases with diagnosis of retinoblastoma in a medical college of Calcutta. Of them, 21 cases were histologically proved to be retinoblastoma. A clinicopathological study of these 21 cases was done. Retinoblastoma patients were between 5.5 and 71 months of age, 57.1% were females, in 90.4% cases there was unilateral involvement and left eye was affected in 68.4% patients. All the patients had negative family history. Majority of the patients (85.7%) presented with leucocoria. Multiple presenting features were present in 5 patients. Aqueous humour cytology revealed malignant cells in 9.5% patients. Imprint cytology of cut end of optic nerve stump was negative for malignant cells in all the cases. Histopathological study revealed presence of Flexner-Wintersteiner rosettes in 23.8%, pseudorosettes in 28.6%, anterior chamber involvement in 38%, choroid invasion in 42.8%, optic nerve involvement in 28.6%, necrosis in 28.6% and calcification in 9.5% cases. After one year of follow-up, none developed any metastasis except one patient who had developed intracranial metastasis.

Orbital rhinosporidiosis.

Rhinospordiosis, a chronic granulomatous disease of the mucocutaneous tissue, is endemic in India and other parts of South East Asia. It commonly affects the upper respiratory tract, but involvement of other sites has also been reported. A 27-year-old male patient reported with features simulating severe obital cellulitis underwent emergency managemt for his systemic problems. Subsequent CT scan and histopathological examination confirmed the diagnosis of rhinoporidiois. Definitive surgical treatment was done to remove the suspected papillomatous mass which led to marked improvement of the patient.