Suravi Mohanty

Segmental dilatation of sigmoid colon in a neonate: atypical presentation and histology

Segmental dilatation of the colon is a rare disorder of colonic motility in children, often presenting with severe constipation in older infants, children, and occasionally adults. It may mimic the commoner Hirschsprung disease clinicoradiologically but differs in that the ganglion cell morphology and distribution are typically normal in the colon. We report a neonate with segmental dilatation of the sigmoid colon who had an atypical clinical presentation and describe certain abnormalities in bowel histology (hypertrophied muscularis propria, nerve plexus, and ganglion cells located within the circular layer rather than the normal myenteric location), for the first time in the English literature.

An unusual case of primary osteosarcoma of the rib in an adult

Primary osteosarcomas are one of the most common malignant bone tumors principally affecting the long bones in children and adolescents. An unusual case of a primary osteoblastic osteosarcoma of the rib in a 42-year-old male is presented here. The patient underwent a wide excision of the tumor and chest wall reconstruction. Although clinically unsuspected in this unusual site, the classic microscopic feature of a ramifying osteoid matrix amidst the tumor cells was diagnostic of an osteosarcoma.

The distal level of normally innervated bowel in long segment colonic Hirschsprung's disease

PurposeThe definitive surgical management of Hirschsprung’s disease (HD) depends on an unambiguous histological diagnosis of bowel aganglionosis, as well as an accurate assessment of its proximal extent from the rectum or ‘leveling’. This study aimed to evaluate and compare the transition zone in rectosigmoid and long segment colonic forms of HD.MethodsOne hundred and fifteen cases of rectosigmoid Hirschsprung’s disease (RSHD) and 20 cases of long segment colonic Hirschsprung’s disease (LScHD) managed from 1998 to 2008 with a consensus prospective protocol were included in this clinicopathological analysis.ResultsIn RSHD, the radiologic transition zone (rTZ)/gross appearance at laparotomy correlated with the histological ‘leveling’ in the majority (96%); the histologic transition zone was also short and predictable. In contrast, the rTZ in LScHD was conclusive in only 53%; overall the radiologic and histological transition zones concurred in only 26%. The histologic transition zone extended proximally for an unpredictable length before normal circumferential innervation was noted.ConclusionsA stringent intraoperative histological evaluation of the colon as outlined is more helpful than radiology to establish the distal level of normally innervated bowel in LScHD and achieve an accurate leveling during a colostomy/pull through.

Actinomycosis in urachal remnants: A rare cause of pseudotumor.

Actinomycosis is a chronic inflammatory condition caused by Actinomyces israeli, a gram positive anaerobic bacterium. It can have a variety of clinical manifestations and can mimic a malignancy. We present one such case of urachal actinomycosis that mimicked a tumor. A 28-year-old man presented with abdominal pain of 20 days duration. Per abdominal palpation revealed a firm mass with ill-defined borders in the suprapubic region. Computed tomography and magnetic resonance imaging scans of the pelvis showed an irregular lesion in the urinary bladder extending to the umbilicus, giving the impression of urachal remnants with inflammation. Peroperatively, an irregular, hard mass measuring 6 × 5 cm, involving the anterior and posterior bladder walls, the appendix, the terminal ileum and sigmoid colon, was seen, which was suspicious for a malignancy. Frozen sections from the mass showed extensive inflammation and a florid fibroblastic proliferation, giving the impression of an inflammatory pseudotumor. The tissue was extensively sampled for paraffin sections and only one of them revealed a colony of Gram, PAS and GMS- positive organisms, conclusive for Actinomycosis. It is important to be aware of this uncommon, yet significant, presentation of a common infectious disease in order to avoid misdiagnosis and over-treatment as a malignancy.

Hemihypertrophy, renal dysplasia and benign nephromegaly.

Hemihypertrophy is associated with malignant visceral abdominal neoplasms in childhood. Benign nephromegaly and nephroblastomas are both known to occur with hemihypertrophy; however, association with renal dysplasia has not previously reported. We describe an infant presenting with recurrent haematuria who had segmental hemihypertrophy, ipsilateral renal dysplasia and contralateral benign nephromegaly. Although debated, renal dysplasia may predispose to and predate malignant change. Rational management and optimal surveillance of renal dysplasia and benign nephromegaly in hemihypertrophy is discussed.

Hirschsprung Disease — Current Diagnosis and Management

Hirschsprung disease is a common cause of neonatal and infantile large gut obstruction. It is characterised by varying extent of contiguous aganglionosis extending from the anorectum proximally. Since its recognition, the diagnosis and management has continuously evolved with advances in histological evaluation and surgical techniques. This article summarizes the current modalities of investigation and optimal surgical management of Hirschsprung disease and concludes with a reference to the Indian scenario.

“The Jelly Belly”: Diagnostic Dilemmas and Current Concepts

BackgroundPseudomyxoma peritonei (PMP) is a rare and poorly understood clinicopathological entity characterized by gelatinous ascites with neoplastic or non-neoplastic mucinous implants in the peritoneum. Although its origin was debated, current evidence in literature favours the appendix as the origin of the disease, over the ovaries. The changing terminologies in the classification of this entity pose diagnostic and management challenges.Case ReportsHerein, we report three cases of PMP in postmenopausal women, their clinical presentation, pathological staging based on the peritoneal tumor deposits and the treatment administered. Two patients recovered uneventfully, while one had recurrence of adenocarcinoma.ConclusionThe rarity of this disease and the diagnostic challenges associated with it are discussed with an emphasis on the current concepts in its origin and management. Appropriate classification and complete removal of the tumor is mandated to prevent disease-related mortality.

Atypical extragonadal germ cell tumors

Aim: To review the experience with the diagnosis and management of extragonadal germ cell tumors (GCT) with a subset analysis of those with atypical features. Materials and Methods: A retrospective chart review of patients of extragonadal germ cell tumors between 2000 and 2010 was carried out. Results: Fifteen children aged 7 days to 15 years (median, 1.5 years) were included. Three had an antenatal diagnosis (one sacrococcygeal, one retrobulbar, one retroperitoneal tumor) and were operated in the neonatal period. The locations were distributed between the retrobulbar area (1), anterior neck-thyroid gland (1), mediastinum (4), abdominothoracic extending through the esophageal hiatus (1), retroperitoneal (4) and sacrococcygeal (4). On histological examination, five harbored immature elements while two were malignant; the latter children received postexcision adjuvant chemotherapy. There was no mortality. At a median follow-up of 4.5 years (6 months to 8 years), 14/15 have had an event-free survival. One immature mediastinal teratoma that recurred locally 7.5 years after the initial operation was excised and adjuvant chemotherapy instituted. Conclusions: Extragonadal GCTs in children are uncommon and occasionally present with atypical clinical, radiological and histological features resulting in diagnostic and therapeutic dilemmas.

Metaplastic carcinoma of breast and neurofibromatosis 1: A rare association

Neurofibromatosis 1 (NF1) patients are generally at higher risk of developing common malignant tumors such as brain and soft tissue tumors. These patients are 5-fold increased the risk of developing breast cancer by the age of 50 years after that the risk remains the same. The most common reported cancer is infiltrating duct carcinoma. We report a case of 61-year-old female with NF1 presented with pain and breast lump for the past 2 months. On mammography, a retroareolar solid cystic lesion measuring 32 mm × 30 mm × 30 mm was noted which was definitive for malignancy. Right-modified radical mastectomy with axillary lymph node dissection was done which revealed a neoplasm composed of glandular and squamous components. Immunohistochemically, these cells were triple negative for estrogen receptor, progesterone receptor, and human epidermal growth factor receptor 2-neu and expressed markers of basal cell differentiation. The final pathological staging was T2N0M0. This is the fourth case report in the English literature with such association. In this case report, we discussed the importance of breast cancer screening in such patients along with a review of the literature.

Spinal teratoma with salivary glandular differentiation

Neurol India 2004;52:87-90. 9. Passacantilli E, Pichierri A, Guidetti G, Santoro A, Delfini R. Surgical treatment of pial cerebellar arteriovenous fistulas with aneurysm of the main feeding artery. Surg Neurol 2006;65:90-4. 10. Tawk RG, Bendok BR, Ali MJ, Getch CC, Batjer HH. Surgical treatment of cerebral arteriovenous fistulas in children. In: Alexander MJ, Spetzler RF, editors. Pediatric neurovascular disease: Surgical, endovascular, and medical management. New York: Thieme Medical Publishers; 2006. p. 90-103.