Subramanian

Antenatal MR diagnosis of urinary hydrometrocolpos due to urogenital sinus

Hydrometrocolpos is cystic dilatation of the vagina and uterus due to congenital vaginal obstruction. It may be secretory or urinary in character and manifests in the neonatal period with abdominal distension. Urinary hydrometrocolpos occurs in patients with urogenital sinus or cloacal anomaly. A rare case of antenatal MR diagnosis of urinary hydrometrocolpos due to urogenital sinus is presented.

MR demonstration of a prostate gland in a female pseudohermaphrodite

We present the rare case of a 14-year-old female pseudohermaphrodite due to congenital adrenal hyperplasia (CAH). MRI revealed the presence of a hypoplastic uterus, ovaries, clitoromegaly, and prostate gland with its typical zonal anatomy. Transrectal sonography also confirmed the presence of a prostate gland.

Aspiration cytology of mesenchymal hamartoma of the chest wall in an infant: a case report.

BACKGROUND Mesenchymal hamartoma of the chest wall is an extremely uncommon lesion of infants. Radiologic features simulate a malignant neoplasm; however, pathologic examination demonstrates an admixture of fibroblasts, benign cartilage and woven bone. There is a paucity of cytologic reports of this rare entity in the available literature. CASE A 5-month-old infant presented with a large right chest wall swelling. Radiologic investigation demonstrated lytic destruction of the right 7th, 8th and 9th ribs with a large soft tissue mass. Fine needle aspiration smears showed lobules of hyaline cartilage and a few spindle cells with abundant chondromyxoid matrix. A cytologic diagnosis of a benign chondroid, possibly hamartomatous lesion was given, which was confirmed as mesenchymal hamartoma on histopathologic examination. CONCLUSION Mesenchymal hamartoma is a rare chest wall lesion that can be diagnosed on cytology, provided that the cytopathologist is aware of this uncommon entity and appreciates the benign cytologic features.

Langerhans cell histiocytosis of pituitary stalk.

A four-year-old male child presented with increased Typically the lesions are round or oval-shaped with wellurine output and excessive thirst for eight months. On defined margins and bevelled edge. Individual lesions examination he had multiple scalp swellings with mild may coalesce giving a geographic appearance to the skull hepatosplenomegaly. Urine specific gravity was decreased suggestive of diabetes insipidus. Skull radiograph revealed multiple well-defined geographic lytic lesions in the calvarium with bevelled margins [Figure 1]. On MR examination of sella, the posterior pituitary bright spot was not seen on T1W image [Figure 2]. The pituitary stalk was thickened and showed uniform enhancement [Figure 3]. The characteristic radiographic feature of skull lesions, MRI findings and hepatosplenomegaly strongly suggested the possibility of Langerhans cell histiocytosis (LCH) which was confirmed on bone marrow biopsy. LCH is a wide-spectrum disease characterized by proliferation of abnormal histiocytes. It encompasses three classical clinical syndromes which in increasing order of severity are: eosinophilic granuloma (solitary bone lesion), Hand-Schuller-Christian disease (skull lesion, exophthalmos and diabetes insipidus) and Figure 2: T1W sagittal image. The bright spot of the posterior Letterer-Siwe disease (multisystemic rapidly progressive pituitary is not seen and the pituitary stalk is thickened. Isointense form). The skull bones are most commonly involved. soft tissue mass is seen in the clivus and sphenoid bone

Peutz-Jeghers syndrome.

Mutations in STK11 are inherited in an autosomal dominant pattern, meaning that children of a mutation carrier each have a 50% risk to inherit the mutation and associated cancer risks. Notably, women and men both have the STK11 gene and have the same chances to inherit and pass down mutations in this gene. Therefore, both sides of the family are important when assessing inherited risk. However, the associated cancers and lifetime risks are different between men and women.

Lingual Thyroid Causing Dysphagia in a Child

Sir, An eight-year-old girl presented with progressive dysphagia of 6 months duration. On clinical examination, there was a small midline mass covered with normal mucosa at the base of the tongue. The thyroid gland was nonpalpable. Systemic examination was normal. He had normal development history. His height and weight were at the 25th and 95th percentile for age respectively. Serum T3, T4 levels were decreased (Free T3-2.5 g/ml and Free T4-0.3 ng/ml) and serum TSH was increased (5 μU/ml). Other biochemical parameters were non-contributory. CT scan of the neck showed well defined intensely enhancing mass in the base of tongue in the midline with absent thyroid in the pretracheal region (Fig. 1a and b). A radionuclide scan was carried out using 99m Tc pertechnetate intravenously. Anterior and right lateral static views of the head and neck region were obtained after 20 min of intravenous injection Tracer accumulation was seen in the lingual region in midline confirming the presence of lingual thyroid (Fig. 1). There was no activity present in the thyroid bed. She was diagnosed as a case of lingual thyroid with hypothyroidism. She subsequently received thyroxine therapy and the clinically symptoms decreased.