Anand Kini

Lipofibromatous Hamartoma of the Median Nerve in Association with or without Macrodactyly

Lipofibromatous hamartoma is a rare tumour-like condition involving the peripheral nerves, particularly the median nerve. It commonly affects the volar aspect of the hands, wrists and forearms of young adults. Most patients present either early with macrodactyly or later with a forearm mass lesion or symptoms consistent with compressive neuropathy of the involved nerve. The clinical and histomorphological findings of five patients with lipofibromatous hamartoma of the median nerve are analysed. The presentation, pathological features and differential diagnosis of neural lipofibromas are discussed along with a brief review of the literature. Of the five cases of lipofibromatous hamartoma, all were seen to involve the median nerve, occurring in four women and one man. Three of these cases had associated macrodactyly which was congenital in two and was seen from childhood in one. Microscopic examination showed fibrofatty tissue surrounding and infiltrating along the epineurium and perineurium. The nerve bundles were splayed apart by the infiltrating adipose tissue. Neural fibrolipomatous hamartoma is a benign condition. Most respond to conservative management with surgical exploration, biopsy and carpal tunnel release to decompress the nerve. Correct diagnosis of this uncommon lesion is important as surgical excision of the lesion may lead to loss of neurological function.

An unusual case of disseminated neuroendocrine tumor presenting with generalized lymphadenopathy.

A 64-year-old male presented to the surgical out-patient department with multiple enlarged lymph nodes in the neck and axillae. As a routine practice in India, this patient was worked up on the lines of generalized lymphadenopathy with a provisional diagnosis of tuberculosis and lymphoma. The report of fine-needle aspiration cytology (FNAC) came as a surprise and on further work-up it turned out to be that the patient had disseminated neuroendocrine tumor from an unknown primary.

Clear cell predominant epithelial-myoepithelial carcinoma of the hard palate — role of immunohistochemistry

Epithelial-myoepithelial carcinoma (EMC) arising from the minor salivary gland of the hard palate is very rare. A 34-year-old lady presented with a nodular swelling in the hard palate. Histopathologically, a diagnosis of clear cell predominant EMC was suggested. Immunohistochemistry demonstrated the biphasic nature of the tumor and confirmed the diagnosis of EMC.

Surgical nuances in the separation of craniopagus twins - Our experience and a follow up of 15 years

Craniopagus twins are conjoined twins fused at the cranium. This is the rarest anomaly seen in conjoined twins and craniopagus twins account for 2– 6% of conjoined twins. Conjoined twins are also extremely rare, with the anomaly seen in about 10–20 subjects per billion births. A female preponderance has been noted. Craniopagus twins can be classified into complete or partial, depending on whether or not they have shared dural venous sinuses. They can be further classified into angular or total depending on the alignment of the inter-twin longitudinal axis. Surgical separation of these cases can be an elective procedure or an emergency, mandated by the death of one of the twins. Surgical separation of craniopagus twins is a complex exercise needing detailed evaluation and planning. For the successful management of these twins, a multi-disciplinary approach involving neurosurgeons, plastic surgeons, anesthetists, radiologists, pediatric critical care specialists and ancillary staff is mandatory. We present a case of partially successful elective separation of partial angular craniopagus twins performed in 2002.The surviving twin was managed conservatively for a cerebrospinal fluid leak. The patient subsequently developed a pseudomeningocele, necessitating re-exploration, excision of the gliotic tissue, and repair of the dura and the overlying scalp flap. The patient has a skull defect for which cranioplasty has been deferred. The child is neurologically intact. The post-operative evaluation and the detailed periodic neurological assessment till date (with a follow up of 15 years) have been presented in this study.

Prof. U. S. Nayak M.S., M.Ch., F.R.A.C.S.

Born in Vijayawada, to Dr. U.V Nayak, Professor of Anatomy at MMC, and Mrs. Sanjeevi Bai, Dr. Nayak graduated in medicine from MMC in 1954 and went on to pursue post‐graduation in general surgery from another prestigious institute, Stanley Medical College in 1959. He was the first batch pupil along with Dr. Mathangi Ramakrishnan, when the legendary Prof. C R Sunderarajan started the first M.Ch. course in plastic surgery in Southern India, at MMC in 1966.

Cytomorphology of Warthin-like variant of papillary thyroid carcinoma

Warthin-like variant of papillary thyroid carcinoma (PTC) is one of the several variants of papillary thyroid cancer. A 36-year-old female came with history of thyroid swelling. Ultra sound showed a solid nodule in the right lobe. Thyroid profile was within normal limits. Fine needle aspiration cytology (FNAC) revealed papillary carcinoma in a background of lymphocytic thyroiditis. Patient underwent thyroidectomy. Histopathology proved it to be Warthin-like variant of PTC with nodal metastasis. This uncommon variant behaves in an indolent fashion. However, our case is unique as it showed nodal metastasis. Cytodiagnosis is challenging with rare reports of cases diagnosed on FNAC. Herein we document cytomorphology of this uncommon variant reported at our institution.