Saumyaranjan Mallick

Pancreatic Extragastrointestinal Stromal Tumors, Interstitial Cajal Like Cells, and Telocytes

CONTEXT The discovery and subsequent ultrastructural characterization of the interstitial Cajal like cells (now called telocytes) in virtually every anatomic sites of the human body, by Laurentiu M Popescu and co-workers, have dramatically improved the understanding the function of these cells and pathogenesis of extragastrointestinal stromal tumors (EGIST). Pancreatic extragastrointestinal stromal tumors (pEGIST), phenotypically similar to pancreatic interstitial Cajal like cells, are extremely rare with an unpredictable biological behavior. OBJECTIVE To review the clinicopathological, radiological, immunohistochemical, and therapeutic outcome data of all reported cases of pEGIST, and highlight the developments in the field of pancreatic interstitial Cajal like cells/telocytes. METHODS A systematic review of English literature (January 2000 to July 2012) was done by using the search engine of PubMed, PubMed Central, Google Scholar, and the Directory of Open Access Journals. RESULTS There have been 19 reported cases of pEGIST during the last decade, over an age range of 31 to 84 years (mean: 56 years) with equal gender predilection ((male:female ratio: 9:10). Preoperative radiological characteristics have been mostly nondiagnostic though these were used, in some, for tissue diagnosis. Majority of pEGIST were localized to pancreatic head (8/19, 42.1%), and 15 of 19 patients (78.9%) were symptomatic at first presentation. The mean size ranged from 2.5 to 35cm (mean: 14 cm). Histomorphological features were that of predominantly spindle cell tumor which consistently expressed c-KIT/CD117 and CD34 by immunohistochemistry, making these two as the most sensitive markers at this site. RESULTS from studies involving discovery on gastrointestinal stromal tumor 1 (DOG-1), the most specific biomarker of GIST/EGIST, has been inconclusive and this was found to be positive in one case only. Neoadjuvant chemotherapy with imatinib mesylate and sunitinib were used in few cases, and genetic analysis of c-KIT proto-oncogene was done in two. By univariate analysis, none of the clinicopathological parameters, except surgical resection with microscopic free margin (R0 resection) (P<0.05), were found to be an important indicators of outcome. CONCLUSION The biological behavior of pEGIST, at present, seems unpredictable which requires indefinite period of follow-up. Large number of such cases with genetic analysis supplemented with immunohistochemistry studies will hopefully throw more light in these tumors.

Cytomorphological and Morphometric Analysis of 22 Cases of Rosai-Dorfman Disease: A Large Series from a Tertiary Care Centre

Background: Rosai-Dorfman disease (RDD) is a rare benign idiopathic histiocytic proliferation. Most commonly reported cases are lymph nodal. Only 30-40% of cases occur in extranodal sites. Although the morphological features of RDD are well documented, there is limited information about the morphometric variations in the histiocytes of this entity. Method: Twenty-two cases of RDD diagnosed on fine-needle aspiration cytology (FNAC) were retrieved from the archives. Both Papanicolaou- and May-Grünwald-Giemsa-stained slides were available for evaluation in all cases. Nuclear area, diameter and histiocyte size were measured taking reactive histiocytes as controls. Results: Among the 22 patients (male/female ratio 3:2; age range 5-55 years, mean 26 years), 3 cases were extranodal and 19 cases were nodal. The nodal sites included cervical, axillary, inguinal and submandibular lymph nodes. The extranodal sites were the retroperitoneum, mediastinum and skin. The most common clinical presentation was enlarged lymph nodes. Cytological features included numerous large benign histiocytes with emperipolesis. All the morphometric parameters were significantly (p < 0.01) higher in RDD histiocytes than in histiocytes in the reactive lymph node. Conclusions: In view of the rarity of the disease and the variable clinical presentation in RDD, accurate diagnosis is necessary. This is the first study to document the morphometric parameters of RDD histiocytes and their comparison with histiocytes in the reactive lymph node.

Delayed isolated port-site metastasis of gallbladder cancer following laparoscopic cholecystectomy: report of two cases.

Laparoscopic cholecystectomy is the standard of care for gallstone disease [1]. The histopathological examination of all gallbladder specimens, though resected for gallstone disease, is recommended in order to detect the underlying gallbladder cancer [2]. The presence of incidentally detected gallbladder cancer following laparoscopic cholecystectomy, in most of the patients (except T1a tumors) warrants a completion radical surgery along with excision of port sites in order to lower the risk of locoregional and port-site metastasis [3]. Port-site metastasis usually manifests early following laparoscopic cholecystectomy, in the presence of underlying gallbladder cancer, and reflects an aggressive behavior of tumor; these patients are mostly not amenable to surgical treatment as they have associated disseminated peritoneal disease. We present two cases of delayed (more than 5 years) port-site metastasis following laparoscopic cholecystectomy; they were not diagnosed with gallbladder cancer at the time of cholecystectomy as their gallbladder specimens were not subjected to histopathological examination.

Invasive rhinosinusitis by Exserohilum rostratum in an immunocompetent child

Exserohilum is a saprophytic fungal pathogen responsible for a wide spectrum of infections in humans. It causes life-threatening acute invasive infections in the immunocompromised individuals, particularly those having haematological disorders. We report a proven case of chronic invasive rhinosinusitis with orbital involvement by Exserohilum rostratum in an immunocompetent child. The patient responded well to endoscopic sinus surgery followed by oral itraconazole. An aggressive surgical approach is required for improving the outcome of patients with invasive infections. A microbiological diagnosis may help in deciding the systemic antifungal agent in fungal rhinosinusitis.

Retroperitoneal and mediastinal follicular dendritic cell sarcoma: report of 3 cases with review of literature

Follicular dendritic cell sarcoma (FDCS) is a rare malignant histiocytic proliferation of antigen presenting follicular dendritic cell. It is an uncommon primary malignancy first described by Monda et al. in 1986. Most commonly reported cases are lymph nodal. Occasional cases occur in extra nodal sites. Here, we describe the clinicopathological features, histomorphology and outcome of three patients with extranodal FDCS along with a concise review of literature on the topic. All three patients were adult females. Two patients were in third decade, and one had age of 50 years. Among the three cases, two cases are presented as retroperitoneal mass and one as mediastinal mass. CT scans revealed heterogeneously enhancing masses. All the cases showed ovoid to spindle neoplastic cells arranged predominantly in whorling, fascicular and storiform patterns with inflammatory infiltrate. Immunohistochemically, the tumor cells are positive for CD21, CD23, CD35 and Clustrin. In view of rarity and variable clinical presentation in FDCS, accurate diagnosis is necessary. Copyright

HIV-associated hematologic malignancies: Experience from a Tertiary Cancer Center in India.

Context and Aim: Data on HIV associated hematologic malignancies is sparse from India. This study attempts to analyze the spectrum and features of this disease at a tertiary cancer center in India. Setting and Methods: Retrospective study from case records of patients registered with a diagnosis of hematologic malignancy and HIV infection between January 2010 and June 2015. Results: Thirteen cases of HIV associated hematologic malignancies were identified, six of them pediatric. HIV diagnosis was concurrent to diagnosis of cancer in 12 and preceded it in one of them. ECOG PS at presentation was >1 in all of them. All patients, except one, had B symptoms. Six of the patients had bulky disease and six are stage 4. Predominant extranodal disease was seen in 67% of them. NHL accounted for 10 of 13 patients and DLBCL-Germinal center was the most common subtype. Mean CD4+ cell count was 235/μL (range, 32-494). HAART could be given along with chemotherapy to 11 patients. Two-thirds of patients received standard doses of therapy. Chemo-toxicity required hospitalization in 58%. CR was achieved in 45% and 36% had progressive disease with first-line therapy. At the time of last follow up, 3 patients were alive with responsive disease, 2 in CR and 1 in PR. None of the pediatric patients were long time responders. Conclusions: These malignancies were of advanced stage and higher grade. Goal of therapy, in the HAART era, is curative. Pediatric patients had dismal outcome despite good chemotherapy and HAART. There is an urgent need to improve data collection for HIV related cancers in India.

Mesothelial/monocytic incidental cardiac excrescence mimicking cardiac tumor.

Mesothelial incidental cardiac excrescence is a non-neoplastic tumor-like lesion commonly occurring in the intracardiac region. The exact etiology is unclear. A 32-year-old woman presented with respiratory distress on exertion. Echocardiography showed severe aortic, mitral, and tricuspid regurgitation, for which triple-valve replacement was performed. A small cardiac excrescence was found over the aortic valve, measuring 0.6 × 0.3 × 0.3-cm, which on microscopy showed features of mesothelial/monocytic incidental cardiac excrescence. This condition is very rare but it must be recognized because it mimics a metastatic malignancy.

Semilobar holoprosencephaly with 21q22 deletion: an autopsy report

Holoprosencephaly (HPE) is the most common forebrain developmental anomaly with a prevalence of 1:16 000 live-births. Possible aetiological agents include environmental factors and genetic defects such as trisomies (13, 18) and deletions (18p, 7q, 2p and 21q). This complex malformation is due to incomplete division of the cerebral hemisphere. The phenotypes of HPE include alobar, semilobar, lobar and midline interhemispheric fusion variants. Craniofacial anomalies occur in 80% of cases. Severely affected babies die in the neonatal period. Here we report an autopsied case of semilobar HPE with pituitary and adrenal agenesis with 21q22 deletion. Additional findings are noted that would help expand the spectrum of 21q22 deletion.

Malignant melanoma of soft parts with osteoclast-rich giant cells: A rare tumour of the jejunum.

Malignant melanoma of soft parts (MMSP), first described by Franz M. Enzinger, is a rare tumour of unknown cell origin. We describe a case of a 45-year-old male who presented with a one-year history of abdominal pain, weakness, and anaemia. Computerised tomography enteroclysis showed a mass in the jejunum that was suggestive of a gastrointestinal stromal tumour. An ulceroinfiltrative lesion measuring 6.5 x 4 x 2cm was identified. Microscopy revealed typical features of MMSP with numerous osteoclasts-like giant cells. The diverse histomorphology and immunohistochemical characteristics of this case involving a rare tumour at a rare site is presented.

Effect of ipsilateral ureteric obstruction on contralateral kidney and role of renin angiotensin system blockade on renal recovery in experimentally induced unilateral ureteric obstruction

Aims: To study, the effects of ipsilateral ureteric obstruction on contralateral kidney and the role of renin angiotensin system (RAS) blockade on renal recovery in experimentally induced unilateral ureteric obstruction. Materials and Methods: Unilateral upper ureteric obstruction was created in 96 adult Wistar rats that were reversed after pre-determined intervals. Losartan and Enalapril were given to different subgroups of rats following relief of obstruction. Results: The severity of dilatation on the contralateral kidney varied with duration of ipsilateral obstruction longer the duration more severe the dilatation. There is direct correlation between renal parenchymal damage, pelvi-ureteric junction (PUJ) fibrosis, inflammation and severity of pelvi-calyceal system dilatation of contralateral kidney with duration of ipsilateral PUJ obstruction. Conclusions: Considerable injury is also inflicted to the contralateral normal kidney while ipsilateral kidney remains obstructed. Use of RAS blocking drugs has been found to significantly improve renal recovery on the contralateral kidney. It can, thus, be postulated that contralateral renal parenchymal injury was mediated through activation of RAS.