Malignant Peripheral Nerve Sheath Tumor Arising from Small Bowel Mesentery: an Extremely Rare Case with Review of Literature.

PURPOSE: Malignant peripheral nerve sheath tumor (MPNST) of small bowel mesentery is a rare tumor. We report a rare case of MPNST of small bowel mesentery in a patient without neurofibromatosis (NF). METHODS: A 50-year-old male, with no features suggestive of NF1, presented to us with complaints of pain abdomen. Contrast-enhanced computed tomography (CECT) of the abdomen revealed a mass in the infrarenal region. On laparotomy, mass was seen to be arising from the mesentery of the jejunum. En-bloc resection of the tumor was done, and histopathological examination was suggestive of malignant peripheral nerve sheath tumor of the small bowel mesentery. RESULT: Patient received adjuvant external beam radiotherapy to a dose of 50.4 Gy to the tumor bed. The patient was planned for chemotherapy but absconded and later came with recurrence. The patient finally succumbed to disease. CONCLUSION: Surgery is the mainstay of treatment. Adjuvant treatment should be based on histopathological report.

Role of intraluminal brachytherapy in palliation of biliary obstruction in cholangiocarcinoma: A brief review.

Surgery is the only curative treatment for cholangiocarcinoma. However, most patients present with advanced disease, and hence are unresectable. Thus, the intent of treatment shifts from curative to palliative in the majority of cases. Biliary drainage with intraluminal brachytherapy is an effective means of relieving the malignant biliary obstruction. In this review, we discuss the role of brachytherapy in the palliation of obstructive symptoms in extrahepatic cholangiocarcinoma.

Solitary fibular metastasis from nonsmall cell lung carcinoma.

Solitary bone metastasis to fibula in patients of lung carcinoma is a rare entity, with only four cases reported in literature. We, hereby, present a case of a 50 year-old-male who was given three cycles of chemotherapy for lung carcinoma with no distant metastasis but presented 2 months later with a fusiform, painful swelling around the knee that was clinically suspected to be inflammatory in nature but proved to be fibular metastasis on cytology. There was no evidence of skeletal metastasis on initial bone scan. He was given palliative radiotherapy for this with symptomatic relief.

Collet-Sicard syndrome secondary to a large glomus jugulotympanicum.

Collet-Sicard syndrome is caused by various neoplastic and non-neoplastic lesions affecting the base of the skull with involvement of IX, X, XI and XII cranial nerves. Paraganglioma accounts for < 1% of all the neoplasms in the head and neck region. They are traditionally considered as benign, slow growing, locally invasive, encapsulated and highly vascular tumors. We report a case of Collet-Sicard syndrome secondary to a large glomus jugulotympanicum in a 45-year-old woman who presented to the emergency department with complaints of recurrent episodes of a fresh bleeding from the left ear for the previous 5 days. She had pain and decreased hearing for the last 3 years and features of multiple cranial nerve palsies. A radiological diagnosis of glomus jugulotympanicum (paraganglioma) was made, which was confirmed by the biopsy tissue. At 6-month follow up, episodes of recurrent bleeding had stopped, but cranial nerve palsies persisted.

Congenital Longitudinal Radial Deficiency in Infants: Spectrum of Isolated Cases to VACTERL Syndrome.

Congenital longitudinal radial deficiency is a rare congenital anomaly and encompasses a spectrum ranging from mild hypoplasia to complete absence of radius. Furthermore known as radial club hand or radial dysplasia, there is variable degree of deficiency along the radial side of the limb. The authors report a case series of four cases; two cases of isolated radial club hand and two associated with other anomalies, including VACTERL syndrome. The rarity of the disease and the need to exclude other associated anomalies are emphasized.

Xanthogranulomatous pyelonephritis: Rare presentation of a rare disease.

Xanthogranulomatous pyelonephritis is a rare chronic renal infection of unknown pathogenesis characterized by replacement of renal parenchyma by lipid filled macrophages frequently associated with an enlarged, non-functioning kidney and an obstructing calculus. We report a case of a 45 year old non diabetic female who presented with gradually enlarging renal mass with extensive retroperitoneal involvement and a non-functioning kidney with no evidence of obstructing stone or fat density and simulating malignancy. She was diagnosed as stage III Xanthogranulomatous pyelonephritis and managed with radical nephrectomy with favourable outcome.

Kirner's Deformity Misdiagnosed as Fracture: A Case Report.

Kirner`s deformity or dystelephalangy is a rare entity which presents with painless, progressive, bilateral radiovolar curving of the terminal phalanges of the little fingers. It is a clinicoradiological diagnosis. Herein, we present a case where the patient was being treated as having a fracture of the distal phalanx because of misdiagnosis of Kirner`s deformity. Given the rarity of the deformity, we believe it useful to present our case report as a contribution to the literature.

Fibromatosis Colli: A Case Report.

Fibromatosis colli or sternocleidomastoid tumor of infancy is a condition of benign proliferation of fibrous tissue within the sternocleidomastoid muscle leading to focal or diffuse enlargement of the sternocleidomastoid muscle and is often clinically associated with torticollis. Radiological imaging especially ultrasound, if performed by an expert radiologist plays an important role in differentiating this benign condition from other causes of neck masses in this age group, thereby preventing unnecessary investigations in a neonate and decreasing parent`s anxiety. We hereby, present a case report of a sternomastoid tumor in a two week old neonate diagnosed using high frequency ultrasound, signifying that every physician should be aware of this clinical entity in a neonate and refer them immediately for ultrasound.

Giant extraosseous Ewing sarcoma of the lung in a young adolescent female--a case report.

Extraosseous Ewing sarcoma is a rare soft tissue tumour that is histologically indistinguishable from the bone Ewing sarcoma. The translocation involving chromosome 22 along with CD 99 expression is pathognomonic and is useful in differentiating from other small round cell tumours. Primary lung involvement by this malignant tumour is very uncommon and up to this date only ten cases have been reported. We report a further case in a 15 year-old-female who presented with a huge lung mass causing an opaque haemithorax.

Role of color Doppler ultrasonography in evaluation of scrotal swellings: pattern of disease in 120 patients with review of literature.

PURPOSE: To determine the value of color Doppler ultrasonography (CDUS) as a routine investigational method for diagnosis of scrotal pathologies. MATERIALS AND METHODS: This prospective observational study (case series) was carried out over a period of 16 months on 122 patients in the age range of 13 to 70 years old, who presented with scrotal swellings. After adequate history taking and examination, CDUS was performed. The diagnosis of the surgeon and that of radiologist were compared with final outcome, which was based on course and outcome of the disease, fine needle aspiration cytology results, and operative findings. RESULTS: The final diagnoses were epididymitis or epididymo-orchitis (46), hydrocele (26), varicocele (16), testicular malignancy (16), orchitis (6), testicular torsion (4), spermatic cord injury (2), hematocele (2), and pyocele (2). Color Doppler ultrasonography accurately diagnosed all cases of epididymitis or epididymo-orchitis, spermatic cord injury, testicular torsion, varicocele, and hydrocele (sensitivity 100% and specificity 100%). Of 16 subjects diagnosed as testicular malignancy on CDUS, only 14 were subsequently found to have malignancy. Two cases of orchitis were wrongly diagnosed as malignancy. Similarly, of 6 patients diagnosed as orchitis, 1 was found to have seminoma (sensitivity 87.5% and specificity 66.7%). Overall sensitivity of CDUS in diagnosing scrotal diseases was 98% while specificity was 66.7%. CONCLUSION: Color Doppler ultrasonography is an excellent, a safe, and reliable method for evaluating patients with scrotal diseases. It aids in diagnosis of testicular tumors and reduces the number of unnecessary exploratory operations. It is especially important in conditions like testicular torsion where immediate diagnosis is required.