Co-occurrence of intracranial Rosai-Dorfman disease and Langerhans histiocytosis of the skull: case report and review of literature.

Rosai-Dorfman Disease involves histiocytic proliferation of the lymphatic system. Extranodal disease involving the central nervous system is uncommon. Furthermore, the combination of this disease entity with Langerhans cell histiocytosis is an even rarer phenomenon that has only recently been highlighted. A young male, who had previously undergone surgical excision of a skull lesion that was reported as Langerhans histiocytosis presented with an intracranial lesion mimicking a meningioma. Histopathology of the lesion was reported as being consistent with Rosai-Dorfman disease and the patient is currently undergoing chemotherapy. This is only the second report of the co-occurrence of Langerhans histiocytosis and Rosai-Dorfman disease in the cranium and intracranial cavity. The possibility that the diseases represent different spectra of the same underlying pathology is one that merits more detailed analysis, especially at the genomic level.

Autochthonous blastomycosis of the adrenal: first case report from Asia.

Systemic endemic mycoses, such as blastomycosis, are rare in Asia and have been reported as health risks among travelers who visit or reside in an endemic area. Adrenal involvement is rarely seen in blastomycosis and has never been reported from Asia. We report the first case of blastomycosis with bilateral involvement of the adrenals in a diabetic patient residing in the state of Arunachal Pradesh, India.

Tricuspid valve mycetoma in an infant successfully treated by excision and complex tricuspid valve repair followed by fluconazole therapy.

BACKGROUND: Fungal valve endocarditis in children is an uncommon and lethal disease. The risk increases with use of central venous catheters (CVC), total parenteral nutrition (TPN), and use of broad-spectrum antibiotics during the neonatal period. Due to high mortality, a combination of surgery and antifungal therapy is usually recommended for treatment. METHODS: Case report and review of the literature. RESULTS: We present a case of an asymptomatic infant with multiple Candida tricuspid valve mycetomas. Complete cure was achieved by combined tricuspid valve repair and fluconazole therapy. We also review 26 cases of tricuspid valve Candida endocarditis in children published in the literature. CONCLUSION: From being uniformly fatal five decades ago to a current survival rate of 64% to 100%, the prognosis of Candida endocarditis has changed dramatically with the use of antifungal therapy alone or in combination with surgery. Our case re-emphasizes the role of valve-sparing debridement with repair of the native valve using autologous pericardium in combination with long-term antifungal therapy as a feasible option in managing tricuspid valve Candida endocarditis.

Verrucous lesions of the oral cavity treated with surgery: Analysis of clinico-pathologic features and outcome.

OBJECTIVE: Verrucous lesions of the oral cavity can be of varied histopathology. The present study evaluates the clinico-pathological features of verrucous lesions of the oral cavity and analyzes the treatment outcomes. MATERIALS AND METHODS: This is a retrospective study of 15 consecutive patients who presented with verrucous lesions of the oral cavity, during the 5-year period from January 2006 to December 2010. Demographic, clinico-pathological features, treatment details, and outcomes were analyzed. RESULTS: Fifteen patients with verrucous lesions of the oral cavity were treated with surgery as the primary modality. The mean age was 62.8 years (range 35-85 years). Wide excision of the primary lesion with adequate mucosal and soft-tissue margins was carried out. Free-flap reconstruction was done in eight patients. All patients remain loco-regionally controlled with good functional speech and swallowing outcome. CONCLUSIONS: Verrucous lesions of the oral cavity are a distinct clinical entity with varied histopathology. A surgical excision with wide margins and appropriate reconstruction is necessary to optimize the disease and functional outcome.

Osteopetrosis: a rare cause of anemia.

Normocytic anaemia is caused either by hypoproliferation of haemopoietic tissue or increased destruction of red cells. Osteopetrosis is a rare cause of anaemia. Infantile osteopetrosis (also called malignant osteopetrosis) is diagnosed early in life. But it is the adult osteopetrosis (also called benign osteopetrosis) which is diagnosed in late adolescence or adulthood that present as anaemia which is difficult to diagnose and treat. Approximately one half of patients are asymptomatic, and the diagnosis is made incidentally, often in late adolescence because radiologic abnormalities start appearing only in childhood. In other patients, the diagnosis is based on family history. Still other patients might present with osteomyelitis or fractures. We are presenting here an unusual case of osteopetrosis which was referred to us for the evaluation of anaemia.