Peripheral type of primitive neuroectodermal tumour arising from the left orbital floor.

Primitive neuroectodermal tumours (PNETs) are rare tumours that originate from primitive neural crest cells. They are usually found in children below ten years of age. Peripheral PNETs (pPNETs) occur in soft tissues of the body, but have the same genetic changes as Ewing's sarcoma of the bone (now called soft tissue Ewing's sarcoma). They commonly present in the thoracopulmonary region, abdomen, pelvis and the extremities. The head and neck regions may also be involved. Our case demonstrates a PNET in the peripheral tissue arising from the left orbital floor and spreading locally to involve the left maxillofacial region, cheek and gum. The incidence of pPNETs is likely to be under-reported in the literature. Recent diagnostic advances, including cytogenetic and immunohistochemical analysis, have allowed these tumours to be distinguished from other small, poorly differentiated round cell tumours such as rhabdomyosarcoma, lymphoma and poorly differentiated synovial sarcoma.

Polymyositis in association with polycythaemia vera.

Inflammatory myopathies are reported to be associated with various malignancies. This association is more commonly observed in cases of dermatomyosis and less frequently in polymyositis. Malignancies commonly reported in association with inflammatory myopathies include non-Hodgkin's lymphoma and ovarian, lung and gastric carcinomas, as well as nasopharyngeal malignancies. Polycythaemia vera as a cause of polymyositis is unknown. We report a case of polycythaemia vera in a female patient who developed polymyositis three years later.

Assessment of the efficacy of pamidronate in ankylosing spondylitis: an open prospective trial.

INTRODUCTION: Bisphosphonates have anti-inflammatory properties in arthritic conditions. This study was conducted to assess the therapeutic potential of intravenous pamidronate in nonsteroidal anti-inflammatory drug (NSAID) refractory/intolerant cases of ankylosing spondylitis (AS). METHODS: A total of 35 NSAID refractory/intolerant AS patients with Bath AS Disease Activity Index (BASDAI) score 4 or above were recruited for the study. Monthly pamidronate infusions (60 mg) were administered to the patients for six months. Treatment outcomes were assessed by comparing baseline values with the values after six infusions using BASDAI, Bath AS Functional Index (BASFI), Metrology Index (BASMI) and Global Score (BAS-G), C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR). An improvement was defined according to the Assessments in Ankylosing Spondylitis (ASAS)-20 and BASDAI-50. RESULTS: 26 patients received all the six infusions. Of these, 22 (85 percent) achieved ASAS-20 and 20 (77 percent) achieved BASDAI-50 responses. Decrements were noted in the mean BASDAI (56.4 percent), BASFI (52.66 percent), BASMI (55.72 percent), BAS-G (66.71 percent), ESR (52.12 percent) and CRP (72.84 percent) after six months. The tender and swollen joint counts of 14 (54 percent) patients with peripheral arthritis were respectively reduced to a mean value of 0.85 and nil, from the baseline of 2.57 and 1.2. Early feel good response was noted in 16 (62 percent) patients within 48 hours of the first infusion. Fever, arthralgia and myalgia were observed in six cases after the first infusion, and in one case, after the second infusion. These symptoms resolved spontaneously within 24 hours. CONCLUSION: Intravenous pamidronate has good efficacy for the treatment of AS.

Rosai-Dorfman disease.

Sinus histiocytosis with massive lymphadenopathy, also known as Rosai-Dorfman disease, is a rare histiocytic proliferative disorder of unknown aetiology. The classic presentation of Rosai-Dorfman disease is massive, bilateral, painless cervical lymphadenopathy. Extranodal disease is also common, often with a particular predilection for the head and neck regions. We report a rare case of Rosai-Dorfman disease with nodal and multiple extranodal manifestations in a 13-year-old girl. The patient had cervical and mediastinal lymphadenopathy, multiple soft tissue swellings of the scalp, bilateral proptosis and goitre due to thyroid gland involvement. She responded to steroids, with remission of the swellings and symptoms. This case is being reported for its rarity, multiple extranodal manifestations and thyroid gland involvement. Thyroid gland involvement in Rosai-Dorfman disease has rarely been reported in the literature.

A cross-sectional study of the clinical profile and aetiological spectrum of pancytopenia in a tertiary care centre.

INTRODUCTION: Pancytopenia is a common haematological problem. It is suspected when a patient presents with anaemia, prolonged fever and a bleeding tendency. Pancytopenia has multiple causes, but the frequency of these causes has been reported in a limited number of studies. The present study was conducted to assess the aetiological pattern, clinical profile and bone marrow morphology of pancytopenia patients. METHODS: A total of 111 adult pancytopenia patients aged 13 to 65 years were studied during a one-year period to determine their clinical features, peripheral blood pictures and bone marrow morphologies. The aetiological pattern was assessed through the relevant investigations in the respective patients. RESULTS: 45.95 percent of the pancytopenic patients had a hypocellular marrow, while 54.05 percent had normocellular or hypercellular marrow. Idiopathic aplastic anaemia (20.72 percent) was the commonest cause of pancytopenia, followed by hypersplenism due to chronic liver disease (11.71 percent). Other important causes were kala-azar (nine percent), megaloblastic anaemia, systemic lupus erythematosus (SLE), infections and drug inducement. Infections such as kala-azar, falciparum malaria and enteric fever, megaloblastic anaemia as well as SLE were found to be treatable and reversible causes of pancytopenia. CONCLUSION: As a large number of pancytopenic patients have a reversible aetiology, early and proper diagnosis may be life-saving.

Oesophageal involvement in mantle cell lymphoma.

The stomach and small intestine are common sites for gut lymphomas, but oesophageal lymphomas are very rare. In mantle cell lymphoma (MCL), although multifocal gut involvement is seen, oesophageal involvement is uncommon. Gut involvement may be primary or secondary to systemic involvement. Multiple lymphomatous polyposis (MLP) is the intestinal form of MCL. Most cases of MLP occur in the elderly, usually over 50 years of age, and the presenting symptoms are abdominal pain, melaena, haematochezia and fatigue. In MCL, tumour cells typically express CD5 and cyclin D1 markers. Our patient presented with generalised lymphadenopathy, dysphagia and rapid weight loss. Upper gastrointestinal endoscopy revealed submucosal polypoid lesions in the oesophagus, stomach and duodenum. Histopathology and immunophenotyping confirmed MCL. Although MLP presenting as a primary MCL of the intestine has been described in the literature, our patient also had multiple intestinal polyposis (including the oesophagus) due to secondary involvement from systemic MCL.

Paraneoplastic palmar fasciitis and polyarthritis syndrome.

Palmar fasciitis and polyarthritis syndrome is a rare paraneoplastic syndrome that has been reported mainly for ovarian cancers. It is thought to be a tumour-associated autoimmune disorder. It is associated with polyarthritis and rapid flexion contractures of hands with palmar nodules due to palmar fasciitis. Similar paraneoplastic features in gastric cancers have seldom been reported. Here, I am reporting a case of paraneoplastic syndrome of metastatic gastric adenocarcinoma with polyarthritis of hands and contractures of multiple joints with asymmetric clubbing (right hand more than left hand) and hyperpigmentation over back of hands and fingers. Hyperpigmentation and asymmetric clubbing have not been reported in literature in palmar fasciitis and polyarthritis syndrome.