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Melanotic neuroectodermal tumour of infancy (MNTI) is an uncommon tumour, predominantly occurring in head and neck, mostly maxilla, but also in skull and mandible. Although a benign lesion, it is known to recur in 15%-27% of cases, and rarely, may undergo malignant transformation. We present a case of a 5-month-old female patient, who presented with a gradually progressive swelling in the right thigh. On imaging, an osteolytic lesion was seen, involving the metadiaphysis of shaft of right femur. A biopsy was performed, on which diagnosis of MNTI was made. MNTI is rarely seen in extremities. To the best of our knowledge, only six cases have been reported in femur, the present case being the seventh. The tumour showed spontaneous regression on follow-up in our patient, which has rarely been described. A knowledge of characteristic morphology and immunohistochemistry is the key to differentiate it from other tumours., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Epithelioid hemangioma (EH) is a rare benign vascular lesion of soft tissue and bone, characterized by endothelial cells with epithelioid or histiocytoid appearance. Though tubular bones, flat bones, vertebra and short bones are common sites for this lesion, the epiphyseal involvement is extremely rare. We present an unusual case of EH of the distal femur in a young boy., Case Report: A 12-year-old boy who had congenital talipes equinovarus of the right foot presented with progressively increasing pain in the right lower thigh for six months. Physical examination revealed muscular atrophy of the right lower limb and a moderately tender swelling in the medial aspect of the right knee without restriction of knee movement. An X-ray revealed an osteolytic lesion, which appeared iso- and hypointense on T1W and hyperintense on T2W MRI images in the distal epiphysis and adjacent metaphysis of the right femur. A radiological diagnosis of chondroblastoma was entertained. The patient was treated with curettage and bone grafting. Histopathology showed a tumor composed of thin-walled arteriolar capillaries lined by large, polyhedral epithelioid endothelial cells with vesicular nuclei, finely distributed nuclear chromatin, and moderate amount of eosinophilic cytoplasm. The endothelial cells were strongly immunopositive for CD34. Mitotic activity was low and the Ki-67 proliferative rate was <2%. A diagnosis of EH was made. EH is a benign lesion and it should be differentiated from its histologically similar malignant counterparts such as epithelioid hemangioendothelioma and epithelioid angiosarcoma as the lesion can be successfully treated with curettage or resection.

Desmoplastic fibroma of bone is an extremely rare locally aggressive primary bone tumour. Diagnosis is tricky and treatment experience limited with tumor surgeons. We studied three rare cases of desmoplastic fibroma arising primarily in bone at unusual sites. Diagnosis was established by radio-histopathologic correlation. Patients were operated with various modalities. Results were evaluated over long term follow-up (6-9 years). The disease was eradicated in all the three cases and no recurrence was seen over long term follow-up. Functional outcome was good and no complications were encountered. Wide resection of the tumor is favoured as the recurrence rates after surgical treatment are high.

Résumé: La maladie de Kimura est une affection chronique inflammatoire rare qui se traduit habituellement par des masses sous-cutanées à la tête et au cou, accompagnées d’adénopathies cervicales. La plupart des cas décrits à ce jour ont été observés en Asie. L’atteinte des membres et les lésions osseuses sont rares. Nous décrivons une observation inhabituelle de maladie de Kimura responsable d’une réaction périostée le long du fémur gauche. Cette maladie bénigne doit faire partie des diagnostics évoqués devant une réaction périostée radiologique. [Copyright &y& Elsevier]