A Rare Case of True Pancreatic Cyst.

Pancreatic cysts can be true or pseudocysts. True pancreatic cysts in children are rare clinical entities. We present a 23-month-old boy with a cystic lesion in the distal body and tail of the pancreas which on histopathology was found to be a rare true congenital simple cyst of the pancreas.

Risk Prediction Scoring System to Predict the Postsurgical Outcomes of Biliary Atresia.

AIM: To find out association between liver function, liver histopathology and outcomes of biliary atresia (BA) following Kasai Portoenterostomy (KPE). MATERIALS AND METHODS: This is a retrospective study of children who underwent KPE at a single institute by single surgeon. The patient records analyzed and data of complete blood counts, liver function tests, coagulation profile and histopathology reports collected. The outcomes recorded as alive and jaundice free, alive but jaundiced, and deceased. Statistical analysis done using SPSS 23. OBSERVATIONS: Total of 148 children operated during January 2000 to December 2018. Of these, 26 matched inclusion criteria. The parameters assessed were percentage of direct bilirubin, ratios of Aspartate transaminase (AST) to Alanine transaminase (ALT); Gamma glutamyl transferase (GGT) to AST; GGT to ALT and Aspartate transaminase to platelet ratio index (APRi). Among histopathology reports, fibrosis grade and bile ductular size noted. Among 26, 16 alive and ten are deceased. Among 16 alive, all are jaundice free. Of the parameters, ratio of AST to ALT, APRi and grade of fibrosis found statistically significant and further analysis showed if AST to ALT ratio < 2.1, APRi < 1.8 and grade of fibrosis < four, irrespective of age at surgery, had 96.2 % probability of successful KPE. Based on these observations, a scoring system and risk prediction model constructed based on Receiver operating characteristic (ROC) curves which are first in BA management. RESULTS AND CONCLUSION: Although numbers are sufficient for statistical analysis, we further intend to validate the scoring system in a prospective trial. BA children can be subjected to risk prediction model and KPE performed in those who have a score less than seven and offered to those with score between eight and 16 out of 20. KEY MESSAGE: The scoring system and risk prediction model can guide in the management and post-operative follow up of children with biliary atresia.

Intrapancreatic accessory spleen: an enigmatic entity.

Solitary hypervascular lesion in the distal body/tail of pancreas in a patient with non-specific abdominal symptoms is a diagnostic challenge. Neuroendocrine neoplasm (NEN) and metastasis from renal cell carcinoma are the most common differentials and intrapancreatic accessory spleen (IPAS) is the rarest of its differential diagnosis. We present, here, a case of a 56-year-old man with a space-occupying lesion in body/tail of pancreas that was preoperatively diagnosed as a NEN based on elevated chromogranin levels and hyperenhancing lesion on contrast-enhanced CT scan. He underwent a spleen-preserving distal pancreatectomy. The final histopathology revealed an IPAS.

Autoimmune Pancreatitis Type II: First Report from India.

Autoimmune Pancreatitis (AIP) presents in two forms - Type I or lymphoplasmacytic sclerosing pancreatitis and Type II or idiopathic ducto-centric pancreatitis (IDCP). AIP II is rare in south Asia and, especially so, in India. Most patients have either Idiopathic Chronic Pancreatitis (ICP) or alcohol related chronic pancreatitis. AIP Type I has been described in India. We herein report a patient who had features of ICP on imaging, for whom surgery was performed to relieve chronic pancreatic pain. However, the pathologic features revealed AIP Type II or IDCP.

Spindle cell oncocytoma of adenohypophysis: Review of literature and report of another recurrent case.

Spindle cell oncocytoma (SCO) of adenohypophysis was first described in 2002 by Roncaroli et al. as a new entity in the tumors originating from adenohypophysis. It was subsequently recognized as a distinct entity in the 2007 WHO classification of CNS tumors and retained in the current updated classification. In contrast to that suggested by the original authors, this tumor does have a potential for recurrence as first described by Kloub et al. and later with many such case reports. This tumor can be confused with other sellar tumors like pituicytomas and pituitary adenoma, both radiologically and histopathologically. However, it is imperative to differentiate these tumors from the above-mentioned differential diagnoses as it certainly has a recurrent potential. To date only 34 cases of SCO have been published in the English literature. Herein we present a rare SCO case with unusually aggressive course in a 64-year-old man, which recurred 4 years after the initial diagnosis.

Reporting the margin in pancreaticoduodenectomies: R0 versus R1.

AIM: This study was conducted to analyze the changes in margin positivity in pancreaticoduodenectomies, on using a standardized protocol, which bread loafs the pancreas and duodenum in the axial plane for adenocarcinomas arising in the pancreatic head, ampulla, terminal common bile duct (CBD), and duodenum, and then to assess whether these tumor subsets involve the margins in different ways. METHODS: The analysis was performed on 70 consecutive specimens, the pre-protocol specimens serving as the control group. RESULTS AND CONCLUSIONS: Tumors originating from the pancreatic head, ampulla, terminal CBD, and duodenum showed a consistent increase in their R1 incidence, post-protocol. Ampullary tumors showed the greatest upward change in R1 positivity. The highest incidence of margin positivity was seen in pancreatic head adenocarcinomas (80%), then distal CBD tumors (60%), and finally the ampullary tumors (39%). In pancreatic head adenocarcinomas, R1 increased from 55% to 80%, distal CBD from 50% to 60%, and ampullary from 17% to 39%. Duodenal adenocarcinomas had no R1 in both pre- and post-protocol groups. The tumors also had different patterns of margin involvement. Ampullary tumors involved only the posterior margin, pancreatic adenocarcinomas involved the superior mesenteric vein (SMV) groove more often than the posterior margin, and distal CBD tumors involved the posterior margin and SMV groove equally. The size of the tumor made a significant difference in pancreatic head carcinomas with tumor size less than or equal to 2 cm, showing an R1 incidence of 38%, while those above 2 cm had an R1 incidence of 68%.

Ileal duplication cyst in the elderly complicated by appendicitis: A rare case report and review of literature.

INTRODUCTION: Gastrointestinal duplication cysts are rare, generally found in infants and young adults. Adult presentation is rare. PRESENTATION OF THE CASE: We present an elderly lady who presented with right iliac fossa pain. Imaging showed a terminal ileal duplication cyst. Laparoscopic right hemicolectomy was done. Histopathology confirmed ileal duplication cyst with reactive appendicitis. DISCUSSION: Surgical resection is deemed appropriate management due to known complications like obstruction, hemorrhage, perforation and malignant degeneration. Resection of only the cyst is adequate in completely isolated cases. However, others require resection of adjoining small bowel. CONCLUSION: We highlight the rare occurrence in elderly and its management laparoscopically.

Interobserver variation is a significant limitation in the diagnosis of Burkitt lymphoma.

CONTEXT: The pathology of classic Burkitt lymphoma (BL) remains a challenge despite being a well-defined entity, in view of the significant overlap with atypical BL and B-cell lymphoma intermediate between DLBL (diffuse large B cell lymphoma) and BL. They are difficult to be segregated in resource-limited setups which lack molecular testing facilities. This is further affected by interobserver variability and experience of the reporting pathologist. AIMS: The aim of our study was to quantitate variability among a group of pathologists with an interest in lymphomas (albeit with variable levels of experience) and quantitate the benefit of joint discussions as a tool to increase accuracy and reduce interobserver variability of pathologists, in the diagnosis of BL in a resource-limited setup. MATERIALS AND METHODS: A set of 25 non-Hodgkin lymphoma cases in which a diagnosis of BL was entertained were circulated to 14 participating pathologist within the Mumbai lymphoma study group. A proforma recorded the morphologic and immunohistochemical features perceived during the initial independent diagnosis followed by a consensus meeting for discussion on morphology and additional information pertinent to the case. STATISTICAL ANALYSIS AND RESULTS: The concordance was poor for independent diagnosis among all the pathologists with kappa statistics (±SE) of 0.168 (±0.018). Expert lymphoma pathologists had the highest (albeit only fair) concordance (kappa = 0.373 ± 0.071) and general pathologists the lowest concordance (kappa = 0.138 ± 0.035). Concordance for morphological diagnosis was highest among expert lymphoma pathologists (kappa = 0.356 ± 0.127). Revision of diagnoses after consensus meeting was highest for B-cell lymphoma intermediate between DLB and BL. To conclude, interobserver variation is a significant problem in BL in the post WHO 2008 classification era. Experience with a larger number of cases and joint discussion exercises such as the one we conducted are needed as they represent a simple and effective way of improving diagnostic accuracy of pathologists working in a resource-limited setup.

Intraoperative parathyroid hormone assay-cutting the Gordian knot.

BACKGROUND: Hyperparathyroidism is treated by surgical excision of the hyperfunctioning parathyroid gland. In case of adenoma the single abnormal gland is removed, while in hyperplasias, a subtotal excision, that is, three-and-a-half of the four glands are removed. This therapeutic decision is made intraoperatively through frozen section evaluation and is sometimes problematic, due to a histological overlap between hyperplasia and the adenoma. The intraoperative parathyroid hormone (IOPTH) assay, propogated in recent years, offers an elegant solution, with a high success rate, due to its ability to identify the removal of all hyperfunctioning parathyroid tissue. AIM: To study the feasibility of using IOPTH in our setting. MATERIALS AND METHODS: Seven patients undergoing surgery for primary hyperparathyroidism had their IOPTH levels evaluated, along with the routine frozen and paraffin sections. RESULTS: All seven patients showed more than a 50% intraoperative fall in serum PTH after excision of the abnormal gland. This was indicative of an adenoma and was confirmed by histopathological examination and normalization of serum calcium postoperatively. CONCLUSION: The intraoperative parathyroid hormone is a sensitive and specific guide to a complete removal of the abnormal parathyroid tissue. It can be incorporated without difficulty as an intraoperative guide and is superior to frozen section diagnosis in parathyroid surgery.

An unusual case of invasive Aspergillus ulcerative tracheobronchitis without involvement of lung parenchyma in a post-renal transplant patient.

We present the case of a 54-year-old male, who presented with respiratory complaints four months after he underwent renal transplantation. Bronchoscopy showed ulcerated mucosa of the left main bronchus and computed tomography (CT) of the thorax showed foci of air within the bronchial wall. A biopsy from the lesion showed septate fungal hyphae, dichotomously branching at acute angles. A locally invasive Aspergillus ulcerative tracheobronchitis with no parenchymal involvement is an important cause of tracheobronchitis in post-renal transplant patients. An early diagnosis and institution of appropriate treatment can improve the outcome. A combination treatment of caspofungin and voriconazole can be considered if patient is not responding to voriconazole alone.

In search of the rainbow: colored inks in surgical pathology.

INTRODUCTION: Although surgical pathologists are aware of the multiple advantages that coloured inks contribute to surgical pathology practice, these coloured inks are not available in India and importing them is not a viable proposition. A systematic search for locally available coloring agents was done, and resulted in identifying specific shades within a popular set of children's hobby colors of a particular brand. They retain their bright distinct colors on paraffin blocks and sections. These paints are available all over India, and are cheap, safe, and easy to use. Coloring gross specimen excision margins with different colors, adds precision to margin examination. It allows three-dimensional microscopic reconstruction of the tumor vis-a-vis its various neighboring anatomic structures. It allows postoperative comparison of tissue planes predicted by preoperative imaging. It maintains orientation of grossed and dissected specimens, enabling the pathologist to re-visit the grossed specimen, if required, and confidently allows further sampling if necessary. AIM: A systematic search for indigenous coloring agents was carried out, which included the dyes used in the histopathology laboratory, gelatin, commercially available paints, including acrylic paints and inks. RESULTS: The study identified specific shades within a brand of acrylic colors that are easily available and simple to use, with good results on microscopic examination. CONCLUSION: Colored inks lend precision to margin examination. A set of easily procurable colors are available in our country, which are easy to use, with distinct bright colors, safe, and reliable.

Kearns Sayre Syndrome--case report with review of literature.

Kearns-Sayre Syndrome is form of rare mitochondrial cytopathy, first described by Thomas P. Kearns and George Pomeroy Sayre in 1958 and is characterized by progressive external opthalmoplegia, cardiac conduction block, pigmentary retinal degeneration, variable number of red ragged fibers on muscle biopsy. It presents before the child reaches the age of twenty. Kearns-Sayre syndrome may affect many organ systems and additional features may include myopathy, dystonia, bulbar symptoms in the form of dysarthria and nasal regurgitation and bilateral facial weakness. Endocrine abnormalities (e.g., diabetes, growth retardation/short stature, and hypoparathyroidism), bilateral sensorineural deafness, dementia, cataracts, and proximal renal tubular acidosis, skeletal muscle weakness (proximal more than distal) and exercise intolerance are additional features. Kearns Sayre Syndrome occurs as a result of large-scale single deletions (or rearrangements) of mitochondrial DNA (mtDNA), which is usually not inherited but occurs spontaneously, probably at the germ-cell level or very early in embryonic development. No disease-modifying therapy is available for Kearns-Sayre syndrome (KSS). Management is supportive vigilance for detection of associated problems. In the future, potential treatment in patients with Kearns-Sayre syndrome may attempt to inhibit mutant mtDNA replication or encourage replication of wild-type mtDNA.

Pathology for the HPB Surgeon.

Optimal care of a patient implies a good professional understanding between all the medical personnel involved in that patient's care. Similarly a basic understanding of the areas where surgery and pathology interact would go a long way, in clarifying the disease process in the patient. This review aims to cover a few topics in liver lesions, FNAC Vs core biopsy, IHC, Margin examination, and frozen sections, in order to improve the communication between these two specialities.

Squamous cell carcinoma of tongue in a renal transplant recipient.

The overall incidence of malignancies in post renal transplant recipients is reported to be approximately 5 to 6%. Malignancies are significant complications after transplantation. Common malignancies include malignancies of the skin and post-transplant lymphoproliferative disorder (PTLD). Squamous cell carcinoma of the tongue is very rare. We present a case of squamous cell carcinoma of the tongue developing nine years after renal transplantation, in a 30-year-old man. He underwent left hemiglossectomy initially and then modified neck dissection. His graft function continues to remain stable.

Retrorectal cyst hamartoma (tailgut cyst) with malignant transformation.

BACKGROUND: Tailgut cyst [retrorectal hamartomas] is an unusual cystic lesion, located in the retrorectal space. It develops from post anal foetal gut remnants, present anterior to the sacrum and posterior to the rectum. A high index of clinical suspicion is required to diagnose this rare tumour. This malformative lesion should be distinguished from teratomas, mullerian cysts, anal gland cysts and duplication cysts of the rectum. The treatment of choice is complete surgical excision. CASE: A tailgut cyst is hereby reported, which had undergone malignant transformation, with development of an adenocarcinoma. Only 16 additional cases of tailgut cysts with adenocarcinoma were recovered, in an extensive search of world literature. CONCLUSION: The identification of this rare entity provides an opportunity to examine this pathological rarity in the context of the few previously reported cases of this type.

Microcystic serous cystadenoma of the pancreas: a report of two cases with one of diffuse presentation.

Microcystic adenoma or serous cystadenoma is an uncommon tumor and accounts for 1-2% of the exocrine neoplasms of the pancreas. Usually unifocal, they present as single, large, well-demarcated multiloculated cystic tumors, ranging in size from 1 to 25 cm. Multifocal variants or diffuse serous cystadenomas are extremely rare. We present 2 cases of which 1 is a diffuse variant affecting the body, tail and part of the neck of the pancreas. In both the patients the tumors were detected incidentally. We highlight on the diffuse variant in view of its rarity and present a review of literature. In this case the entire body and tail of the pancreas was spongy replaced by multicystic lobules and hyalinized fibrocollagenous stroma. The cysts were lined by low cuboidal glycogen containing bland cells. Such a unique presentation wherein the entire body and tail of the pancreas is replaced with multiple cysts is a diffuse presentation of microcystic adenoma and a search through literature revealed only 7 such cases among the 15 cases with multifocal presentation reported.