Munita Bal

A rare case of primary gastric plasmacytoma: An unforeseen surprise

Primary plasmacytoma of the gastrointestinal tract is a rare entity. We report a case of a primary gastric plasmacytoma in a 57-year-old man who presented with upper-gastrointestinal bleeding. Endoscopy showed a nodular gastric mass with central umblication. Histological examination of the gastrectomy specimen revealed a monoclonal lambda-chain extramedullary plasmacytoma. Further staging was found to be negative for multiple myeloma. As other more common pathologic processes at this site may also be endowed with numerous plasma cells, awareness of this entity and distinction using immunohistochemistry are extremely crucial. Because systemic disease ultimately develops in many patients with localized plasmacytoma, such patients should be followed closely for the appearance of clinical, biochemical, and roentgenologic evidence of multiple myeloma.

Are we achieving the benchmark of retrieving 12 lymph nodes in colorectal carcinoma specimens? Experience from a tertiary referral center in India and review of literature.

INTRODUCTION The number of lymph nodes (LNs) retrieved from a specimen of colorectal carcinoma may vary. Factors that can possibly affect LN yield are age of the patient, obesity, location of the tumor, neoadjuvant therapy, surgical technique and pathologists handling of the specimen. AIM The aim of our study is to look at lymph node retrieval from colorectal cancer (CRC) specimens in our hands and review the literature. MATERIALS AND METHODS From May 2010 to January 2011, a total of 170 colorectal carcinoma cases were operated in our institute. Type of the surgeries, lymph node yield was looked at. RESULTS There were 103 (60.6%) males and 67 (39.4%) females. The commonest age group was 50-59 years (30.6%). The surgeries included 107 surgeries for rectal carcinoma (63%) and 63 surgeries for colonic carcinoma (37%). Sixty six (38.8%) cases had received preoperative chemoradiotherapy, whereas 104 (61.2%) cases were without adjuvant therapy. The total lymph node positivity (metastatic disease) was 44.7% .The overall mean lymph node yield was 12.68 (range 0-63; median 11). The mean lymph node harvest in the age group < 39 was 15.76 whereas, the lymph node harvest in the group more than 39 years old was 11.90. ( statistically significant; P=0.03). The mean lymph node yield from specimens of rectal cancers (10.30) was lower than the mean lymph node yield from specimens for colonic cancers (16.71);( statistically significant, P<0.01). There was also statistically significant difference between the mean LN yield in chemoradionaiive cases (14.63) and in the cases where neoadjuvant therapy was received, (9.59); P<0.01. CONCLUSION Pathologist while assessing a specimen of CRC should aim to retrieve a minimum of 12 LN. Surgical expertise and diligence of the pathologists remain two main alterable factors that can improve this yield. Neoadjuvant or preoperative radiotherapy can yield in less number of nodes.

Pathology of Gallbladder Carcinoma: Current Understanding and New Perspectives

Gallbladder carcinoma is a rare and highly lethal malignancy. It stands out from amongst the other GI tract malignancies for its unique epidemiological profile, proclivity for female gender, definitional ambiguities, ability to escape early diagnosis, and absence of effective treatment. Pathobiology of gallbladder carcinoma continues to remain poorly understood. Recently, better characterization of the precursor lesions and elucidation of underlying molecular pathways has enhanced our understanding of gallbladder tumorigenesis. Proposal of a unified terminology and evolving consensus in classifying gallbladder pre-invasive neoplasia offers hope of better assimilation of rare data from diverse parts of the world. Identifying biomarkers and cancer specific cellular targets that will pave the way for novel therapeutic approaches for gallbladder carcinoma is urgently needed. In this review we delve into the epidemiologic, genetic and pathologic characteristics of this enigmatic disease with a special focus on the recent advancements in the field of gallbladder pathology.

Adenocarcinoma of the Rectum-A Composite of Three Different Subtypes With Varying Outcomes?

INTRODUCTION Adenocarcinoma of the rectum has been classified by the World Health Organization into various histologic subtypes. We analyzed the effect of the histologic subtype (classic, signet ring cell, and mucinous) on the clinical outcomes of patients with rectal cancer. We hypothesized that clinicopathologic outcome measures such as tumor margins, tumor regression grade, recurrence rate, and survival would vary with the histologic subtype. MATERIALS AND METHODS We conducted a retrospective analysis of a prospectively maintained database. All patients with stage I-III rectal adenocarcinoma were included. RESULTS From May 2010 to August 2013, 273 patients underwent curative resection. Both mucin-secreting variants were more common in younger patients and presented at a more advanced stage. Also, 54% and 48% of those with signet ring cell carcinoma (SRCC) and mucinous adenocarcinoma (MAC) had node-positive disease compared with the rate in the classic variant (30%). Circumferential resection margin positivity was 24% with MAC and 19% with SRCC compared with 4% with the classic variant. Disease-free survival for those with the classic and mucinous variants was 38.5 and 37.4 months, respectively. In contrast, it was 28.6 months in the SRCC group. The overall survival did not differ significantly. CONCLUSION Rectal adenocarcinoma presents as a spectrum of disease, with progressively worsening outcomes from classic to MAC to SRCC. These aggressive variants might warrant more aggressive resection. These data from the Indian subcontinent differ from the published data from Western countries.

Solid pseudopapillary tumor of the pancreas: ‘Experiences’ and ‘Lessons’ at a tertiary‐care oncology center

Solid pseudopapillary tumor of the pancreas (SPT) is a rare and fascinating entity of elusive histogenesis and unpredictable biology. It has a peculiar proclivity to afflict young females and involve the pancreatic body‐tail region. Cytology diagnosis of these rare neoplasms remains a challenge. We analyzed the cytology features of all SPT cases diagnosed on fine needle aspiration cytology (FNAC) from 2003 to 2009 along with their histopathology slides. Nineteen consecutive cases were diagnosed as SPT on FNAC. Fifteen out of nineteen cases were confirmed as true SPT on histopathology. Amongst the true SPT, all except one occurred in females. Age ranged from 14 to 50 years. Pseudopapillae bearing stout branches terminating in bulbous tips and enclosing transgressing vessels, separated from a collar of tumor cells by a clear zone of myxohyaline coat were pathognomonic of SPT. Singly dispersed monomorphic tumor cells with bland chromatin formed the second diagnostic component of SPT. Nuclear grooves and hyaline globules were in addition helpful in segregating SPT from its close differentials. In four cases diagnosed as SPT on FNAC, histopathology revealed a different final diagnosis (one case each of paraganglioma, extragastrointestinal stromal tumor, metastatic papillary renal cell carcinoma and inflammatory myofibroblastic tumor). Conversely, one case of SPT had been erroneously diagnosed as neuroendocrine tumor on FNAC. Six cases (40%) developed metastasis; commonest site being liver. In conclusion, cytology in conjunction with clinico‐radiologic findings plays a key role in making a correct diagnosis. Awareness of unique cytomorphological features is important in distinguishing this tumor from its diverse mimics. Diagn. Cytopathol. 2013.

Angioimmunoblastic T-Cell lymphoma: A critical analysis of clinical, morphologic and immunophenotypic features

BACKGROUND Angioimmunoblastic T-cell lymphoma (AITL), a subtype of peripheral T-cell lymphoma (PTCL), is characterized by unique clinical and biological features. Its diagnosis remains a challenge as clinical presentation as well as pathologic findings are frequently misleading. MATERIAL AND METHODS We retrospectively analyzed the clinical, morphological and immunophenotypic spectrum of 17 cases of histologically proven AITL. RESULT The mean age was 54 years and male to female ratio was 2.4. Common clinical features included generalized lymphadenopathy (60%), hepatomegaly (70%), splenomegaly (50%), anemia (80%) and polyclonal hypergammaglobulinemia (100%). Microscopically, three architectural patterns; pattern I (6%), pattern II (41%) and pattern III (53%) were observed. Bone marrow infiltration was seen in 60% cases and 30% cases revealed plasmacytosis. Absence of follicles, polymorphous infiltrate, extra-follicular follicular dendritic cell (FDC) proliferation, high endothelial venules (HEV) prominence and neoplastic T-cells were the diagnostic features of AITL. CD10 positivity (47%), clear cells in the background (59%) admixture with large size CD20+ B-immunoblasts (35%) and bone marrow plasmacytosis (50%) were common observations. CONCLUSION Awareness of various morphological and immunophenotypic complexities of AITL and distinction from reactive adenopathies and other types of lymphomas that mimic AITL is underscored in this study.

Stage IV lung cancer: Is cure possible?

Reporting a case, 53 years old male with stage IV Nonsamall cell lung cancer in view of cytologically proven malignant pleural effusion. Usually the management of stage IV lung cancer is with palliative intent where the patient receives palliative chemotherapy along with palliative radiotherapy and surgery if required. Most of the data on curative management of oligometastatic non-small cell lung cancer includes patient with adrenal metastasis and some reports with brain metastasis. There is scarce literature on the surgical management of stage IV lung cancer with pleural effusion.

Angiosarcoma of the scalp associated with Xeroderma pigmentosum

Xeroderma pigmentosum (XP) is a rare autosomal recessive genodermatosis associated with hypersensitivity to ultraviolet light due to defects in Deoxyribonucleic acid (DNA) repair. These patients have more than a 1000-fold increased risk of developing skin cancers. Although multiple cutaneous malignancies are common, the simultaneous occurrence of angiosarcoma and basal cell carcinoma is a rare phenomenon. We report a case of a 25-year-old male with XP with angiosarcoma scalp and basal cell carcinoma of face and occiput and discuss the treatment of this aggressive neoplasm with a review of the literature pertaining to it.

Primary intestinal T cell lymphomas in Indian patients - In search of enteropathic T cell lymphoma

OBJECTIVE This series of six intestinal T cell lymphomas (ITCL) attempts to document enteropathy-associated T cell lymphoma (EATCL) in India. MATERIALS AND METHODS A total of six ITCL were selected from 170 gastrointestinal lymphomas in last 10 years. RESULTS The cases studied included EATCL (4), ITCL with a CD4 positive phenotype (1) and ITCL NK/T cell type (1). Of the four EATCL, two occurred in the ileum, one in right colon and one in duodenum. In three EATCL cases, there was history of celiac disease or lactose intolerance and enteropathic changes were noted in the adjacent mucosa. These tumors had CD3+/CD8+/CD56 (+/-)/CD4-/ Granzyme B+ immunophenotype. One EATCL was monomorphic small cell type (type II EATCL) with a CD3+/CD8-CD56+/CD4-/ Granzyme B+ phenotype. EBER- ISH (Epstein Barr virus coded RNAs- in situ hybridization) revealed positive tumor cells in ITCL NK/T cell type and in bystander cells in three EATCL. CONCLUSION ITCL are rare in Indian patients but do occur and comprise a mixture of the enteropathic and non-enteropathic subtypes.

Gastrointestinal Neuroectodermal Tumor: a Diagnostic Dilemma

Malignant gastrointestinal neuroectodermal tumor (GNET), a rare soft tissue sarcoma, is a recently described distinct clinicopathological entity. With only a few cases reported in literature till date, there is limited knowledge about the behavior as well as diagnosis of this tumor. GNET mimics several other tumors and hence presents as a diagnostic challenge to clinicians and pathologists alike. We report a case of gastrointestinal neuroectodermal tumors with liver metastasis.