Rhinoscleroma in a child

Rhinoscleroma is fairly common in the Northern and Central parts of India. The disease may present itself with many unusual features and may mimic many other diseases, specially Carcinoma. A patient with unusual features of Rhinoscleroma is being reported.     

Multiple myeloma in a child

A 12-year-old girl with the diagnosis of multiple myeloma is described. She presented with a nasopharyngeal mass which was histologically found to be a plasmacytoma. Serum immunoelectrophoresis revealed an IgA-kappa M-protein (4.9 g/dl). There were approximately 20% atypical plasma cells in a bone marrow biopsy specimen. The diagnosis was further supported by immunohistochemical demonstration of cytoplasmic monoclonal IgA-kappa in the tumor cells of both the nasopharyngeal and bone marrow biopsies. The patient was treated with chemotherapy for 1 year, at which time she became refractory to treatment, based on serum IgA levels. Five months after cessation of therapy, she continues to exhibit a significant objective response, remaining clinically well with a stable, elevated serum IgA level.     

Modern aspects of child urology

Basing on the results of modern investigations applied preoperatively, postoperatively and for 20 years of follow-up in more than 36,000 children with urogenital diseases, the author believes that further progress of childhood urology should advance in the scope of general urology. He proposes basic principles underlying decision making on the treatment of urological diseases in different age groups. It is shown that many open and endoscopic interventions used in the adults are converted into pediatric urological practice disregarding pathogenetic and compensatory features in urological diseases in children. Basing on specific features of the course of compensatory processes in various age groups of children, new terms of corrective operations are proposed.     

Glomus tumour in a child

Glomus tumours are uncommon neoplasms. Very rarely do they present in the paediatric age group. We describe a case of bilateral glomus tumours in a 13‐year‐old girl that were successfully treated with radiotherapy. The patient remains well 8 years after completion of treatment.     

Parotid gland carcinoma in a child

A rare case of undifferentiated carcinoma of the parotid gland in a child is reported.     

Dermatofibrosarcoma protuberans occuring in a child

Dermatofibrosarcoma protuberans (DFSP) usually occurs in adults, however it can also occur in infancy and childhood. Diagnosis of DFSP in children is quite difficult given the rarity of the tumor and its variegated appearance. The behavior of this neoplasm is of borderline or intermediate malignancy with a high rate of recurrence but limited metastatic potential. We present the case of an eleven-year old boy who presented with a slightly raised, not tender lesion on his right shoulder. It had appeared two years before as a red-brown plaque and since then it had gradually grown to a nodule. An excisional biopsy was performed, and the diagnosis of DFSP was made based on the histological and immunohistochemical findings.     

Orbital apex syndrome in a child

A 8-year-old male presented with visual loss, diplopia, ptosis, pain behind the left eye, facial numbness and vomiting of one week duration. The ophthalmological, neurological and radiological examination showed a lesion of the left orbital apex with extension into the cavernous sinus. Examination of the nose and paranasal sinuses did not reveal any abnormality. Transnasal Endoscopic orbital decompression was performed and inflamed granulation tissue found in the orbital apex was removed. Microbiology showed fungal elements which on culture grew Aspergillosis flavus. Antifungal therapy with new generation oral drug (voriconazole) resulted in complete resolution of symptoms. Relevant literature is reviewed and discussed.     

Four primary tumors in one child

A child is described who, following surgery and irradiation for malignant choroid plexus papilloma at the age of 11 months, developed three further tumors: A benign myxofibroma of the cranium at the age of 3 1/2 years, an undifferentiated anaplastic tumor of his clavicle at 7 1/2 years, and a chondroblastic osteogenic sarcoma of his pelvis at the age of 12 years. There was no apparent genetic predispositions to malignancy. The latter three neoplasms may have originated either in, or at the edge of, the original radiotherapy field, suggesting that the child has a particular susceptibility to the oncogenic potential of radiotherapy.     

Leukemia in a child with Wilms' tumor

A case is described which illustrates an acute non-lymphocytic leukemia following multimodal control and apparent cure of Wilms' tumor. Literature cases are reviewed, and therapeutic inadequacies according to present protocols and their importance for the subsequent development of secondary leukemia are discussed.     

Pneumothorax preceding pulmonary blastoma in a child

Serial plain radiographic, ultrasound and CT findings of an unusual case of pulmonary blastoma are described with a review of the literature.     

Fibrolipoma of the nasopharynx in a child

Besides angiofibroma, benign tumours of the nasopharynx are in general rare. A true lipoma of the nasopharynx is rarely reported. Fibrolipoma in a child is a very rare tumour and is hence reported.     

Paraneoplastic papilloedema in a child with neuroblastoma

Non-metastatic neurological disease complicating neuroblastoma is well recognized. Gross papilloedema in the absence of intracranial disease as initial manifestation of neuroblastoma is reported in adults. We report for the first time a case of bilateral papilloedema in a child with neuroblastoma in the absence of intracranial disease and hypertension.     

Recurrent adrenocortical carcinoma in a child.

An 8-month-old girl presented with clitoromegaly, cushingoid features and a large abdominal tumour. Ultrasonography (US) and computed tomography (CT) of the abdomen revealed a tumour of the left suprarenal gland, 12x11x7 cm in size. Serum levels of cortisol, testosterone and DHEA-S, and urinary extretion of 17-ketosteroids and 17-hydroxycorticoids were increased. Complete removal of the tumour was accomplished through a transabdominal approach. The diagnosis of adrenocortical carcinoma was confirmed histologically. Three months after the first operation, a recurrent tumour of the left renal hilus, 23x15 mm in size, was identified by US and verified by aspiration biopsy. The tumour was removed by the transabdominal route. In this report, we discuss the diagnosis and the treatment of this rare disease.