Pallavi Aga

Imaging Spectrum in Soft Tissue Sarcomas

Imaging plays an important role in detection, diagnosis as well as pre and post operative management of patients with soft tissue sarcomas. Soft tissue sarcomas are generally a diagnostic dilemma needing the complimentary use of both radiology and pathology for their accurate diagnosis. In this review article, we have tried to highlight the important facts about the various imaging modalities available as well as the recent advances in the field of radiology.

Renal cell carcinoma: impact of mode of detection on its pathological characteristics.

Objective: Data correlating mode of presentation of renal cell carcinoma (RCC) with pathological prognostic factors is sparse from India. We compared RCC presenting incidentally with those presenting symptomatically with respect to pathological prognostic factors and assessed whether this could serve as a decision making resource for diagnosing small and more favorable tumors. Materials and Methods: The data were reviewed for 328 patients operated for renal tumors between January 2000 and October 2008 at our institute. The pathological factors (tumor size, stage, grade, histopathological type) in relation to the mode of presentation were analyzed according to 1997 TNM criteria. Statistical analysis was performed via the chi-square (Fisher exact) and Mann - Whitney U test. The statistical significance level utilized was P < 0.05. Results: Among the patients assessed, 93 (28.4%) had incidental diagnosis and 235 (71.6%) had symptomatic presentation. Sex and side distribution was not significantly different in the two groups. Mean tumor size was 5.75 ± 2.73 cm in incidentally detected RCC (IRCC) and 9.32 ± 3.70 (P < 0.001) in symptomatic RCC (SRCC). Stage I and II tumors were significantly greater in IRCC than SRCC (p<0.001 and 0.005 respectively) whereas stage III and IV tumors were significantly less in IRCC than SRCC. There was a predominance of higher grade tumors in SRCC, 50% being higher grades (Fuhrmans grade III and IV) in SRCC than 28.1% in IRCC (P = 0.003). There were 4 tumors with collecting duct histology in SRCC and none in IRCC. Sarcomatoid differentiation was present in 14 and 1 patient in SRCC and IRCC respectively. Conclusion: Incidental detection of renal carcinoma as compared to symptomatic tumors is lower in India as compared to western world. Incidental tumors have significantly favorable pathological prognostic factors. Our results might form a basis for further studies on how to pick RCC at an earlier stage.

A new classification of inferior vena cava thrombus in renal cell carcinoma could define the need for cardiopulmonary or venovenous bypass.

Introduction: Although the level of inferior vena cava (IVC) thrombus governs the type of surgical approach, there is no consistency in reporting the levels of IVC thrombus in the literature. This prospective study illustrates a simple three-level classification based on the need for clamping hepatoduodenal ligament and venovenous or cardiopulmonary bypass. Materials and Methods: Between January 2010 and June 2014, 30 patients of renal mass with renal vein and/or IVC thrombus were treated after classifying the IVC thrombus into three levels on the basis of need for clamping the hepatoduodenal ligament. After excluding renal vein thrombi, level I was described as thrombus located caudal to the hepatic vein. Level II included all retrohepatic, suprahepatic infradiaphragmatic or supradiaphragmatic thrombi reaching till the right atrium. Atrial thrombi were categorized as level III. Level I and II thrombi were managed without venovenous or cardiopulmonary bypass. Level III thrombus required cardiopulmonary bypass. Results: Of 26 patients with thrombus, 13 had level I thrombus. Of eight cases with level II thrombus, three were retrohepatic, three were suprahepatic infradiaphragmatic and two were supradiaphragmatic. All were removed successfully. Of five patients with level III thrombus, three were operated with cardiopulmonary bypass while the remaining two patients were too sick to be taken up for surgery. The median hepatoduodenal ligament clamp time was 10 min. One patient with level II thrombus had transient liver enzyme elevation. Conclusion: Renal vein thrombus should not be categorized as level I thrombus. Level II thrombus, irrespective of its relation to the diaphragm, could be managed without venovenous or cardiopulmonary bypass.

Primary mesenteric lymphangioma in a young adult with intestinal malrotation and 'counter-clockwise barber pole sign'

Mesenteric lymphangiomas are uncommon benign lesions that usually occur in isolation but rarely may be associated with gastrointestinal (GI) malrotation. Malrotation may cause chronic and recurrent volvulus leading to chronic venous congestion and lymphatic engorgement. Interference with lymphatic drainage may result in formation of a lymphangioma or a chylous mesenteric cyst. In our case, mesenteric lymphangioma was present with partial malrotation of small and large bowel. There was no radiological and surgical evidence of volvulus, favouring the hypothesis that lymphangioma may be a primary congenital association of GI malrotation. Another interesting finding was the counter-clockwise twisting of the superior mesenteric vein around the superior mesenteric artery which has been reported only seven times in the literature.

Multiple neural tube defects in a child: A rare developmental anomaly

Background: The presence of multiple neural tube defects (NTDs) is a rare entity. Published literature shows not more than 10 case reports. Such cases contradict the well-established “zipper model” of neural tube closure and support “multi-site closure model.” Case Description: We are reporting a unique case of multiple NTDs in a 5-month-o ld female child. Occipital encephalocele, dorsal meningomyelocele, Split cord malformation (SCM), and tethered cord were present in this case. Conclusion: This case report further substantiate the “multisite closure model,” however, more research work on human neuro-embryology is needed to overcome the controversies of neural tube closure.

Swyer–James–MacLeod syndrome with ipsilateral herniation of hyperinflated hyperlucent lung

Swyer–James–MacLeod syndrome is characterised by unilateral hyperlucency on chest radiograph with small or normal-sized lung on the affected side and compensatory hyperinflation of opposite lung. Hyperinflation of the affected lung is a very rarely reported entity. An adult female patient, who presented with exertional breathlessness and diagnosed to have hypoplastic left pulmonary artery with hyperlucent, hyperinflated and herniated left lung is described.

Case report: MR spectroscopy in pantothenate kinase-2 associated neurodegeneration

We report a case of a 13-year-old girl with Hallervorden-Spatz disease (HSD) or pantothenate kinase-2 associated neurodegeneration (PKAN). HSD is a rare neurodegenerative disorder, which is characterized by a rapidly progressive extrapyramidal syndrome, dementia with optic atrophy, and retinal degeneration. It is associated with accumulation of cysteine-iron complex in the globus pallidi and substantia nigra. The MRI “eye of the tiger” sign is the characteristic. MRI spectroscopy is also characteristic. It shows markedly decreased NAA/Cr values in the globus pallidi and substantia nigra with increased mI/Cr values that suggest of gliosis.

Giant right atrial aneurysm presenting as right heart failure.

Idiopathic aneurysmal dilatations of the right atrium are rare anomalies. We report one such case of a young man presenting with fatigue, abdominal distension, pedal oedema, unremarkable cardiac examination except for raised jugular venous pressure, an electrocardiogram showing normal sinus rhythm with right bundle-branch block, and an radiograph of the chest showing cardiomegaly. The echocardiographic examination revealed a giant right atrium with low pressure tricuspid regurgitation. The computed tomography confirmed the findings of two-dimensional echocardiography. He was put on medical treatment and remained symptomatically controlled on follow-up.

Giant unilateral hydrocele "en-bisac" with right hydronephrosis in an adult: A rare entity.

Abdominoscrotal hydrocele consists of two large sacs; the abdominal and scrotal, both are connected via the inguinal canal. The diagnosis is made by clinical examination and can be confirmed with ultrasound scan. Surgical treatment is considered mandatory since spontaneous resolution is extremely rare. Herein, we report a case of giant unilateral hydrocele en-bisac who presented with spontaneous rupture of the sac.

MRI in isolated dextrogastria with eventration of the right hemidiaphragm with associated mesentero-axial volvulus

Isolated dextrogastria is the rarest of all visceral transpositions and usually coexists with eventration of the right hemidiaphragm. We herein report a unique case of isolated dextrogastria with eventration of the right hemidiaphragm along with mesentero-axial volvulus. Such a case has not been reported in young children. As the barium study was suboptimal, MRI helped by exquisitely depicting the anomaly without radiation risk to the child.