Schwannomas are one of few truly encapsulated neoplasms of human body. These are composed of Schwann cells. In general, schwannomas have a predilection for the head, neck, mediastinum, and retroperitoneal regions, sciatic nerve involvement is rare. The patient presents with pain in hip region radiating along the lateral side of leg, may be associated with other neurological signs and symptoms. Treatment of symptomatic schwannomas consists of marginal excisional resection with emphasis on identification of the nerve fascicles and excision of the tumor stalk. Here, we present a case of sciatic schwannoma in a young female, which was diagnosed on histopathology.

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Kimura's disease and angiolymphoid hyperplasia with eosinophilia (ALHE) together form a spectrum of chronic inflammatory disorders of unknown etiology with similar and overlapping histological features. We report a case of Kimura's disease in a young Indian girl with its cytohistological correlation. The cytological features of these inflammatory disorders are fairly specific and in an appropriate clinical setting, a cytological diagnosis of Kimura's/ALHE is possible. However, it may not be possible to differentiate between the two based on cytology alone.

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Acute erythroid leukemia is a very rare entity in children. Here is a case of erythroleukemia with complex cytogenetics in a child aged 2 years. On immunophenotyping, CD45 versus side scatter (SSC) demonstrated blast population (29%) with intermediate SSC and moderate CD45 expression. The myeloid nature of blast population was confirmed by moderate expression of cytoplasmic myeloperoxidase, CD117, CD13 and CD33. Another population of cells (28%) with low to intermediate SSC and negative CD45 expression revealed dim expression of CD235a (64%) indicating lysis resistant abnormal erythroid progenitors. Conventional cytogenetic analysis by G-banding revealed complex cytogenetics.

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Xanthogranulomatous inflammation is rare, and though it has been described in many organs, most commonly it occurs in the kidney and gall bladder. Xanthogranulomatous appendicitis is a very rare phenomenon. Clinical findings and imaging modalities are not sufficient for definite preoperative diagnosis. Clinical and radiological resemblances of these lesions to neoplastic processes often warrant excision. A 40-year-old female presented with pain in the right lower quadrant of the abdomen, loss of weight, nausea, and abdominal distension since 15 days. Ultrasonography and computed tomography of the abdomen and pelvis suggested neoplastic lesion. Surgical resection of the ileum, ileocecal junction with appendix, and part of transverse colon were done. Diagnosis of xanthogranulomatous appendicitis was made on histopathology examination.

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Carcinosarcoma (CS) of the uterus is rare and accounts for 1–2% of all uterine malignancies, occur commonly in postmenopausal women. These are highly aggressive tumors with poor prognosis and often present at advanced stage. Tamoxifen (TAM) has been known to increase the incidence of endometrial carcinoma from 1 to 2 cases per 1000 women/year and of uterine sarcoma from 0.04 to 0.17 cases per 1000 women/year. TAM has weakly estrogenic properties that can produce endometrial cell proliferation and, consequently, TAM use increases the risk of endometrial cancer by approximately two- to three-fold. Currently, no consensus is present regarding the management of Uterine CS. However, surgery plays an important role in the management along with chemotherapy (CT) and radiotherapy as an adjuvant. We report a case of a woman who developed malignant mixed mullerian tumor of uterus after taking TAM for 6 years as adjuvant hormonal therapy for breast carcinoma.

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Paraneoplastic cerebellar degeneration (PCD) is a rare presentation of Hodgkin's Lymphoma (HL) manifests as acute/sub-acute nature. We report a case of 21 yr old male presented with acute cerebellar signs along with underlying HL.MRI brain was normal. CSF study was unremarkable. Patient was treated with six cycles of chemotherapy followed by radiotherapy. Neurological manifestations remarkably improved along with complete resolution of underlying HL. Anti-cancer therapy of underlying HL is the main strategy of treating associated PCD.

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Acute myeloid leukemia (AML) presenting as massive pericardial effusion is extremely rare and very few cases have been reported. We present the case of a 16-year-old boy who presented with massive pericardial effusion due to leukemic infiltration. He was diagnosed as AML M1. He received chemotherapy, achieved remission, and pericardial effusion resolved. After 15 months, he relapsed with the reappearance of pericardial effusion.

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Carcinoid tumors of testis are very rare and account for <1% of all testicular neoplasms. Clinically, they can be diagnosed only when there is a metastatic spread or presence of carcinoid syndrome. Mostly, they are diagnosed on histopathology. We report a case of primary carcinoid tumor of testis in a 34-year-old male without associated carcinoid syndrome. Patient underwent radical orchidectomy and is doing well on follow-up. Testicular carcinoid tumors, though rare, should be considered in differential diagnosis when evaluating a testicular tumor. They carry a good prognosis; however, long-term follow-up is necessitated due to potential for delayed metastasis.

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Synovial sarcomas are aggressive malignant soft-tissue tumors that often involve large joints of the lower extremities. Cricopharynx is a rare site for primary synovial sarcoma with no case reported until date. We report a case of a 45-year-old male presented with progressive dysphagia and hoarseness of voice. On examination by computed tomography revealed a soft-tissue mass in the cricopharyngeal region with involvement of posterior wall of the laryngeal framework. The endoscopic biopsy was suggestive of biphasic synovial sarcoma, which was confirmed by immunohistochemistry. The patient underwent surgery followed by radiotherapy and chemotherapy. Despite multimodality, management patient developed lung metastasis, and survived for 10 months only. There are no available guidelines for treatment of such rare tumors, and each case needs to be reported.

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Solid pseudopapillary neoplasm (SPN) of the pancreas is a rare neoplasm of low malignant potential that mostly affect young women. These tumors are of unclear pathogenesis, are slow growing, and can become considerably large before causing symptoms. Complete resection is curative in most cases. Metastases are frequently amenable to resection. Favorable prognosis with long-term survival has been shown even in patients with metastatic disease. We report a case of SPN of pancreas in a 15-year-old female.

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Adult peripheral neuroblastoma represents less than 1% of all malignant primary tumors of peripheral nerves. We report a young adult who presented with swelling over the left wrist with left epitrochlear and axillary lymph nodes. Incision biopsy of the swelling was suggestive of malignant small round cell tumor. On immunohistochemistry, cells were positive for synaptophysin, chromogranin and nonspecific enolase and negative for leucocyte common antigen, cytokeratin, CD99 and myogenin. Urinary vanillyl mandelic acid and homovanillic acid levels were elevated. The patient received 8 cycles of chemotherapy (OJEC). Reassessment positron emission tomography-computed tomography scan showed a complete metabolic response at the primary site and partial response at left axillary lymph nodes. The patient underwent axillary lymph node clearance followed by radiotherapy to the tumor bed and lymph node regions. The patient could not afford autologous haematopoetic stem cell transplant and was started on isotretinoin maintenance. He is on follow-up for 12 months and disease free.

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Pleomorphic sarcoma, not otherwise specified/undifferentiated pleomorphic sarcoma (UPS) is one of the most common sarcomas occurring between the ages of 50 and 70 years. We present a case of a 50-year-old male with a paratesticular mass clinically diagnosed as Fournier's gangrene due to ulceration and necrosis of the overlying scrotal skin. Histopathological examination finally led to the diagnosis of UPS.

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Cytokeratin and epithelial membrane antigen (EMA) are usually included in the first panel of immunomarkers used to differentiate metastatic carcinoma from lymphoma in cases presenting with enlarged lymph nodes. While carcinomas are cytokeratin and EMA positive, most lymphomas are negative for the above. However, recently few cases of cytokeratin positive lymphomas have also been reported. Here, we describe a very rare case of cytokeratin positive anaplastic large cell lymphoma (ALCL) masquerading as a poorly differentiated carcinoma. Simultaneously, we also discuss the differential diagnosis and difficulty in differentiation from metastatic carcinoma in such a scenario. Review of literature shows that this is probably the first case report of anaplastic lymphoma kinase negative-ALCL seen in a young adult.

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Myxomas can be found in heart, skin, sub-cutaneous tissue, and centrally in the bone but are rare in the oral cavity. Fibromyxoma (FM), the locally invasive benign tumor of the jaw, though rare in the general population, occurs with excessive frequency among young female patients. The tumor develops from the ecto-mesenchymal portion of the tooth germ and shows an inactive effect of nests of odontogenic epithelium on mesenchymal tissue or as a direct myxomatous change in fibrous tissue; hence, it is also called as odontogenic myxoma. The tumor usually occurs in the posterior mandible and constitutes around 3–6% of all odontogenic tumors. Clinical, radiographic, histopathologic, and immunohistochemical investigations play an important role in the accurate diagnosis of FM. A complete surgical excision with long term follow-up is essential for successful management. This clinical study emphasizes the unusual variations in presentation of FM of the jaws.

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Symplastic hemangioma (SH) represents degenerative changes occurring in preexisting hemangioma that can mimic sarcomas and pseudosarcomas. Around eight cases of SH have been reported so far in the literature indicating its rare presentation. On the other hand, multinucleate cell angiohistiocytoma (MCAH) is believed to be a reactive inflammatory process rather than a true neoplastic process. Around 80 cases of MCAH have been reported so far. Both these entities share common histopathological features such as the presence of vascular channels, bizarre cells, inflammatory stroma, and multinucleate giant cells making them to masquerade sarcoma. Hereby, we report a case of a tiny swelling over the dorsum of hand in a 30-year-old female, with a clinical diagnosis of hemangioma. The histopathological examination revealed poorly circumscribed vascular lesion with bizarre cells, multinucleated giant cells scattered against the dense inflammatory background. We considered differential diagnoses of MCAH, SH, and dermatofibroma. We also considered malignancies in differential diagnoses such as hemangioendothelioma, liposarcoma, and metastatic nodule due to the presence of bizarre cells. This case report reemphasizes the presence of atypical histological features that represent degenerative changes occurring in benign tumors which can masquerade malignancies. We discuss the histological features that will help to differentiate SH and MCAH.

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Primary adenocarcinoma of fallopian tube is one of the rarest and accounts for about 1% of all gynecological malignancies. We are presenting this rare case of the primary fallopian tubal carcinoma. A 60-year-old female patient with para 2 visited the Gynecologic and Obstetric Department with the complaints of white discharge and abdominal pain since 4 months duration. Radiological study suggested hydrometra with polypoidal growth in the left adnexa suggestive of malignant lesion. The patient underwent the total abdominal hysterectomy with bilateral salpingo-oopherectomy along with the omental resection. On histopathology diagnosed as the primary adenocarcinoma of the fallopian tube and was confirmed by immunohistochemistry with cytokeratin-7 positivity.

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In the stomach, benign tumors are not common. Even when they do occur, they are usually considered as premalignant lesions with a risk of transformation to malignant ones. Tubulovillous adenomas of the stomach are rare. However, these tumors are being diagnosed with increasing frequency due to more widespread use of endoscopy. We present an unusual case of a large tubulovillous adenoma of the stomach. The partial gastrectomy specimen showed a large polypoidal growth at the greater curvature of the stomach. The patient is symptom-free, after 2 years of follow-up.

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Background: Identification of JAK2V617F mutations has led to a significant development in our understanding of the pathogenesis and therapy of BCR-ABL1 negative myeloproliferative neoplasms (MPNs). However, not all cases of BCR-ABL1 negative MPNs carry JAK2V617F mutations. The present study was undertaken with an aim to standardize the real-time quantitative polymerase chain reaction (PCR) for the detection of JAK2V617F mutations and to find the prevalence of Janus kinase 2 (JAK2) mutations in MPNs in the Indian scenario. Materials and Methods: Real-time quantitative PCR was used to detect the JAK2V617F mutation. Standardization of the detection procedure was carried out using recommended guidelines to ensure the accuracy and reproducibility of results. Forty-nine patients of BCR-ABL1 negative MPNs were included in the study. Results: The JAK2V617F mutation was detected in 63.3% patients of BCR-ABL1 negative MPNs. On classification of these BCR-ABL1 negative MPNs, JAK2V617F mutation was detected in 78.3% patients with polycythemia vera (PV), 62.5% patients with essential thrombocythemia (ET), and 44.4% patients with Primary myelofibrosis (PMF). Conclusion: Role of detection of JAK2 mutations in BCR-ABL1 negative MPN has begun to be described and can be used in the diagnosis of PV, ET, and PMF along with other criterias. In future, it may be suitable for treatment monitoring and prognostication.

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Background: The latest World Health Organization classification incorporates extensive description of immunophenotype of the neoplastic cells while describing chronic lymphoproliferative disorders (CLPDs). The present study was undertaken with an aim to identify and compare the roles of flow cytometry (FCM) and immunohistochemistry (IHC) as modalities of immunophenotyping in the diagnosis of CLPDs. Materials and Methods: Thirty untreated cases of CLPDs were enrolled in the study. Twenty eight cases of B-CLPD were divided into two groups - chronic lymphocytic leukemia (CLL) (21 patients) and non-CLL (7 patients). Peripheral blood/bone marrow aspirate samples were analysed by FCM using various panels of monoclonal antibodies. Immunohistochemical analysis of bone marrow biopsies obtained from these patients was also performed. Results: Panel A of monoclonal antibodies comprising CD5, CD23, CD22, surface membrane immunoglobulin (SmIg), FMC7 and Panel B comprising CD5, CD23, CD22, SmIg, FMC7, CD79b were useful (P < 0.01 and <0.001 respectively) while Panel C comprising CD5, CD23, SmIg, FMC7 and CD79b was not found to be useful in distinguishing CLL from non-CLL (P > 0.05) The concordance rate between FCM and IHC ranged from 80% to 100% for all comparable immunological markers. In all cases of CLPDs, we propose a screening panel comprising 9 markers including CD19, CD5, CD23, FMC7, CD10, CD20, CD3, kappa and lambda, which are important for specifying the lineage (B or T), to differentiate CLL from non-CLL group and for deciding the secondary panel. Conclusion: Scoring system using CD5, CD23, CD22, FMC7, CD79b, and SmIg is useful in differentiating CLL from non-CLL cases. Concordance rate of FCM and IHC in CLPDs is 93.3%. Using a panel comprising CD19, CD5, CD23, FMC7, CD10, CD20, CD3, kappa and lambda, a diagnosis of CLL, mantle cell, and follicular lymphoma, the three most common CLPDs can be made. Secondary panels for diagnosis of hairy cell leukemia and T-cell CLPD should be utilized.

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Gestational trophoblastic diseases include complete and partial molar pregnancy, invasive mole, placental site trophoblastic tumor, and choriocarcinoma. Gestational choriocarcinoma is one of the most malignant form of a group of tumors. Although choriocarcinoma has a very high propensity to metastasize to various sites including brain it also has a very high cure rate. Our study represents the case of choriocarcinoma developed after primary invasive mole which was treated successfully with combined surgical and medical management. We now present a course of gestational trophoblastic disease transforming from benign to a malignant condition in due course of management. We are presenting a case of a 26-year-old female who came to us with molar pregnancy which turned out be invasive mole on histopathological examination and converted into choriocarcinoma in due course of treatment even after methotrexate chemotherapy.

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Oral squamous cell carcinoma (OSCC) is one of the most feared human cancers due to its aggressiveness and high mortality rate. One of the reasons for its treatment failure is thought to be related to the presence of a subpopulation of cells within the tumor called cancer stem cells (CSCs) which are highly tumorogenic, capable of self-renewal, and have the ability to differentiate into cells that constitute the bulk of tumors. Recent evidence suggests that CSCs are especially, resistant to conventional therapy and are the "drivers" of local recurrence and metastatic spread. Research in the recent years has been aimed at the application of stem cell biology to clinical medicine, particularly its role in the evolution and metastasis of tumors. Several stem cell markers are known to be expressed, mainly in the basal layers of oral mucosa which are necessary for tissue homeostasis. These specific markers for CSCs have been investigated in head and neck SCC in the hope of developing a deeper understanding of the role of CSCs in oral cancer pathogenesis, diagnosis, and developing newer therapeutic strategies. This review aims at presenting CSC and their biomarkers with a special emphasis on their role in oral tumorogenesis and OSCC metastasis. It also aims to explain the potential role of these CSC in improving the diagnosis, prognosis, and treatment of OSCC patients.

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The incidence of oral cancer remains high and is associated with many deaths. Several risk factors for the development of oral cancer are now well known, including smoking, drinking and consumption of smokeless tobacco products. Genetic predisposition to oral cancer has been found in certain cases but its components are not yet entirely clear. A number of genomic lesions accompany the transformation into oral cancer and a wealth of related results has appeared in recent literature. Tumor suppressor genes can be grouped into 3 categories like caretaker genes, gatekeeper genes, and landscaper genes; the classification schemes are evolving as medicine advances, learning from fields including molecular biology, genetics, and epigenetics. Tumor suppressor genes in oral cancer have been analyzed a lot in many studies. This review highlights the important areas about tumor suppressor genes in oral cancer and provides an overview on the understanding of these genes.

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