Bing-Neel syndrome: A rare complication of waldenstrom macroglobulinemia.

ABSTRACT: Central nervous system involvement is a rare complication of Waldenstrom macroglobulinemia (WM) accounting for 1-2% cases. This syndrome is called Bing-Neel syndrome (BNS) after the people who first described it in 1936. Although WM has a good prognosis, with the onset of this syndrome, most patients fare poorly with a high mortality rate. A 77/male, a known case of WM, presented with left upper limb weakness of 2 weeks duration. Magnetic resonance imaging brain showed diffuse pachymeningeal thickening along bilateral frontoparietal convexity. Biopsy showed lymphoplasmacytic lymphoma favoring a diagnosis of BNS. The patient was started on chemotherapy (rituximab + ibrutinib). At 3 months following diagnosis, the patient was admitted with Gram-negative septicemic shock and failed to recover from it. BNS is a rare complication of WM, associated with poor prognosis and an aggressive clinical course. It can occur during the course of treatment of WM, as was seen in this case. Accurate diagnosis with appropriate treatment plays a crucial role in patient management.

Desmoid fibromatosis-a diagnostic dilemma.

PURPOSE: Desmoid fibromatosis in head and neck is infrequent and poses a significant challenge to the clinicians due to its non-specific characteristics. METHODS: This case report focuses on a 69-year-old male who presented to a tertiary healthcare center in Karnataka, India with a swelling in the oral cavity. RESULTS: Despite initial suspicions of malignancy based on clinical examination and findings on computed tomography imaging, subsequent histopathology and immunohistochemistry revealed an unexpected finding. CONCLUSION: The case highlights the importance of clinical suspicion and histopathological evaluation as well as the need for greater awareness to facilitate early diagnosis and appropriate management of desmoid fibromatosis. We also present a literature review of varied presentations of desmoid tumors afflicting various subsites of the head and neck.

Colorectal Carcinoma With Sarcomatoid Components: Report of 15 Cases and Literature Review of an Exceedingly Rare Carcinoma Subtype.

Colorectal carcinoma with sarcomatoid components (which includes so-called carcinosarcomas and sarcomatoid carcinomas) is a rare subtype with 50 reported cases in the literature and overlapping criteria with undifferentiated carcinoma. We collected and described 15 cases from 10 men and 5 women, with a mean age of 66 years. Symptoms included abdominal pain and gastrointestinal bleeding. Most tumors presented in the rectosigmoid region, with a mean size of 8.2 cm. The sarcomatoid component, on average, represented 58% of the tumors and took many forms, including spindled (10 cases), anaplastic (9 cases), and rhabdoid (3 cases); one case showed osteoid matrix. Tumor budding was usually high, and tumor-infiltrating lymphocytes were usually low. The sarcomatoid component was keratin-positive in 10 cases. One case showed loss of mismatch repair protein expression, and 2 cases showed SMARCA4 loss (1 also with SMARCA2 loss). Molecular testing identified mutations in KRAS (n=1), NRAS (n=2), BRAF (n=2), APC (n=1), and TP53 (n=1) in a few cases. Tumors often presented at advanced stage, with 11 cases pT4, 9 cases with nodal metastases, and 7 cases with distant metastases. Follow-up was available for 10 cases (median: 2 months), with 2 alive without disease, 3 alive with disease, and 5 dead. Our findings roughly corresponded with those in previously reported cases. Colorectal carcinoma with sarcomatoid components is rare and aggressive, with a poor prognosis for many patients. We suggest that spindled cells, anaplasia, heterologous elements, and/or a component with definable sarcomatous lineage be used to distinguish colorectal carcinoma with sarcomatoid components from undifferentiated carcinoma.

Multifocal intraosseous pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma: A rare presentation of an uncommon tumor.

ABSTRACT: Pseudomyogenic hemangioendothelioma (PHE) is an uncommon mesenchymal neoplasm of intermediate malignant potential showing endothelial differentiation. Around 20 cases of primary osseous PHE have been reported to date. A 16-year-old boy presented with complaints of pain in his right leg. Imaging revealed multifocal intramedullary and cortical-based lytic lesions involving long and small bones. Microscopic examination revealed plump, spindled cells arranged in fascicles and admixed "epithelioid" and "rhabdoid" cells sans vasoformative areas. By immunohistochemistry, the lesional cells were reactive for AE1/AE3, CD31, Erg, Fli1, and SMA, while immunonegative for CD34, myogenin, and S100. Nuclear expression of the INI1/SMARCB1 protein was retained. PHE is a rare entity, more so as a primary osseous lesion; therefore, awareness of the presence of this entity in the bone is the key to making a diagnosis. We discuss its clinicopathological features, differential diagnosis, and an attempt a short review of the literature.

Anterior abdominal wall metastasis following curative resection and chemoradiation of rectal cancer masquerading as a desmoid tumour: A clinical conundrum.

Desmoid tumour of the anterior abdominal wall (rectus sheath) commonly occurs in women post abdominal surgery. Metastasis from colorectal cancer to the anterior abdominal wall, on the other hand, is rare and produces a complex management dilemma. This Case study presents a 57-year-old woman who received a curative laparoscopic low anterior resection and adjuvant chemoradiation in 2013. Seven years later, she presented with an asymptomatic anterior abdominal wall lump. Clinically, the lump appeared to be a desmoid tumour. A wide local excision of the lump was carried out and the final histopathology showed a metastatic lesion (adenocarcinoma). With adjuvant chemotherapy, the patient is now disease-free and doing well. A possibility of distant metastasis must be kept in mind for all patients, even when they have undergone curative resection with adjuvant chemoradiation for colorectal cancer.

Primary Adrenal Epithelioid Sarcoma in a Child: A Case Report with Literature Review.

Introduction; Epithelioid sarcoma is a malignant mesenchymal neoplasm with evidence of epithelial differentiation. All the cases reported in the solid organs are of "proximal type" occurring in adults. We report a primary epithelioid sarcoma arising in the adrenal gland of a young male. Case report: An 11-year-old male patient presented with right loin pain. Imaging revealed a 10.8 × 10.8 × 13.5 cm complex cystic mass with obscured right adrenal gland. Clinical and radiological studies did not reveal metastases. Histologic features were those of proximal type epithelioid sarcoma with extensive central necrosis. Immunohistochemistry showed strong positivity for pancytokeratin, vimentin, and CD34. Nuclear expression of SMARCB1 (INI-1) protein was lost. Conclusion: Proximal type of epithelioid sarcoma can arise from solid organs such as the adrenal.

Primary cervical lymph nodal leishmaniasis.

Leishmaniasis, an endemic disease in several parts of India, is a parasitic disease caused by protozoa and transmitted by the sandfly. Although cutaneous and visceral varieties are common, isolated lymph nodal involvement is extremely rare, and the diagnosis is often delayed owing to its uncommon presentation and lack of awareness of this possibility. We present a 72-year-old woman with asymptomatic right facial lymphadenopathy which on biopsy revealed leishmaniasis and responded well to drug therapy.

Rectal actinomycosis mimicking malignancy.

Abdominal actinomycosis is a chronic suppurative infection caused by Actinomyces species. The ileo-cecal region is most commonly affected, while the left side of the colon is more rarely involved. The infection has a tendency to infiltrate adjacent tissues and is, therefore, rarely confined to a single organ. Presentation may vary from non-specific symptoms and signs to an acute abdomen. We present a 56-year-old male who underwent a colonoscopy for complaints of bleeding per rectum that showed an ulcero-proliferative lesion at the recto-sigmoid junction that seemed like malignancy. Multiple biopsies done were reported this lesion to be Actinomycosis. He responded well to medical management that avoided a major surgical procedure.

Histopathological Spectrum of Meningiomas with Emphasis on Prognostic Role of Ki67 Labelling Index.

BACKGROUND & OBJECTIVE: Meningiomas are the most frequently encountered primary non-glial tumors of the central nervous system (CNS). The Ki67 labelling index (Ki67LI) is a proliferation marker that may prove useful in determining the histological grade. This study aims at: 1) Studying the frequency, grade and histomorphological spectrum of meningiomas, 2) Evaluating 20 histological parameters and determining its utility in grading meningiomas and 3) Comparing the Ki67LI in the various subtypes and WHO grades. METHODS: The cases of meningiomas diagnosed in our Department from June 2009 to May 2014 were included. The clinical details, grade and 20 histological parameters: mitosis, vesicular nuclei, macronucleoli, nuclear pleomorphism, scattered bizarre nuclei, hypercellularity, sheeting, lymphocytes, small cell change, foam cells, ossification, necrosis, papillary change, lipidization, psammoma bodies, vascularization, brain invasion, dural invasion, bone invasion and other soft tissue invasion were recorded for each case. The average and highest Ki67LI was recorded as percentage and number per high power field. RESULTS: A total of 175 cases of meningioma were included: grade I (145), grade II (30). Atypical histological features like hypercellularity, sheeting, etc. were common in grade II tumors. Increased vascularity, lymphocytes and psammoma bodies were common in grade I tumors. Ki67LI (highest) ranged from 1-6% in grade I and 5-12% in grade II tumors. CONCLUSION: Among different methods showing mitotic activity, Ki67% (highest) was the most statistically significant LI in differentiating grade I and grade II tumors. The median Ki67% (highest) was 4% for grade I and 7% for grade II tumors.

Mucinous Tubular and Spindle Cell Carcinoma: A Review of Histopathology and Clinical and Prognostic Implications.

Mucinous tubular and spindle cell carcinomas are rare kidney tumors with generally indolent behavior. As the name suggests, classic histomorphology reveals bland spindle cells, tubules, and mucinous stroma. Uncommon histologic features include mucin-poor stroma, high nuclear grade, cellular pleomorphism, and presence of necrosis. Rare cases can show aggressive growth and distant metastasis. Mucinous tubular and spindle cell carcinoma has characteristic chromosomal abnormalities and the molecular signature remains the same, irrespective of the varied histomorphology.

Anxiolytic activity of ultra-diluted Aconitum napellus on Wistar rats

BackgroundAnxiety disorders are highly prevalent that affect millions of people worldwide. Homeopathic system is gaining popularity due to its efficacy in chronic diseases. Ultra-diluted Aconitum napellusis a homeopathic medicine used by clinicians for treatment ofacute anxiety.AimObjective of this study is to test the efficacy of Aconiteon experimental anxiety models of Wistar rats. Methodology18 Female Wistar rats were dividedinto Control, Anxiety, andAnxiety + Aconite(ANX+ACO) groups. Acute stress was induced by restraining the rats for sixhours daily for fivedays in Anxiety and ANX + ACO groups. After fivedays of stress,a single dose of Aconite200cH was given to ANX + ACO group through oral gavage, whereas control and anxiety group rats were maintainedwith a normaldiet. Following this, behavioral assessment was done. Histological sections of the hippocampal area weretaken and stained with Hematoxylin and Eosin and also Cresyl violet stain. ResultsIt was observedthat the Aconitetreated group had morenumber of entries and also the percentage of time spent in the open arm was higher than the stressed anxiety group. Histological study of the brain tissue procured from all three groups was analyzed. ConclusionIt was inferred that ultra-diluted homeopathicAconitehas decreased anxiety in Wistar rats which were subjectedto acute restrainstress. However,the decrease was not statistically significant to deduce Aconiteas an anxiolytic.(AU)

Oncocytic Variant of Medullary Thyroid Carcinoma - A Case Report.

Medullary thyroid carcinoma (MTC) is a rare tumor arising from parafollicular C-cells. The oncocytic variant of MTC is an extremely rare diagnosis, with less than 20 cases reported. Here we present the case of a 36-year-old male patient with complaints of neck swelling and dysphagia. On fine needle aspiration cytology (FNAC), a Hürthle cell neoplasm was suggested. Finally with histopathology and immunohistochemistry (IHC), a diagnosis of MTC oncocytic variant was established. This tumor can be easily misdiagnosed for any thyroid Hürthle cell lesions. An accurate diagnosis is important because MTC has different treatment protocols, and its oncocytic variant is expected to be associated with poorer patient survival. Thus, the oncocytic variant of MTC is a difficult diagnosis on FNAC. Histopathology and rel- evant IHC markers are necessary for a correct diagnosis.

Hematolymphoid neoplasms in effusion cytology.

BACKGROUND: Hematolymphoid neoplasms (HLNs) presenting as body cavity effusions are not a common finding. They may be the first manifestation of the disease. A diagnosis on effusion cytology may provide an early breakthrough for effective clinical management. AIMS: Study the cytomorphology of HLNs in effusion cytology, determine common types, sites involved and uncover useful cytomorphologic clues to subclassify them. MATERIALS AND METHODS: Twenty-four biopsy-proven HLN cases with malignant body cavity effusions and 8 cases suspicious for HLN on cytology but negative on biopsy are included in this study. Effusion cytology smears were reviewed for cytomorphological features: cellularity, cell size, nuclear features, accompanying cells, karyorrhexis, and mitoses. RESULTS: Diffuse large B-cell lymphoma (37%) was the most common lymphoma type presenting as effusion followed by peripheral T-cell lymphoma (25%). Pleural effusion (75%) was most frequent presentation followed by peritoneal effusion (20.8%). Pericardial effusion was rare (4.1%). The common cytologic features of HLNs in effusions: high cellularity, lymphoid looking cells with nuclear enlargement, dyscohesive nature, and accompanying small lymphocytes. Mitosis and karyorrhexis were higher in high-grade HLNs when compared to low-grade HLNs. Myelomatous effusion showed plasmacytoid cells. Very large, blastoid looking cells with folded nuclei, high N: C ratio, and prominent nucleoli were seen in leukemic effusion. CONCLUSION: HLNs have characteristic cytomorphology and an attempt to subclassify them should be made on effusion cytology. Reactive lymphocyte-rich effusions cannot be distinguished from low-grade lymphomas based on cytomorphology alone. Ancillary tests such as immunocytochemistry, flow cytometry, and/or molecular techniques may prove more useful in this regard.

Toxic Effects of Different Doses of Cyclophosphamide on Liver and Kidney Tissue in Swiss Albino Mice: A Histopathological Study.

BACKGROUND: Cyclophosphamide (CPA) is an anti-cancer drug, used in chemotherapy. This is a toxic drug which targets the cancer cells and also the normal cells of the body. The original compound is inactive in vitro and exercises its biologic action through metabolites, chiefly phosphoramide mustard. The objective is to study the harmful effects of this drug on liver and kidney tissues. METHODS: To study the effect of cyclophosphamide on histology of liver and kidney, 40 adult male mice were taken and divided into two groups: control and test. Those in the test group were injected with the drug at doses of 100, 200, 250 mg/kg body weight. They were then sacrificed on day 7, 28 and 42. The liver and kidney tissue was processed, sectioned and stained with Haematoxylin and Eosin. RESULTS: Pathological changes were seen in the tissue within 7 days in high doses and after 28 days in low doses. As the dosage and the days administered increased, the changes were prominently seen and widespread. Pathology ranging from mild infiltration to necrosis and finally cytolysis were seen in liver and kidney tissue. CONCLUSION: Our study has demonstrated the effect of a progressive increase in dosage of cyclophosphamide in albino mice, and pathological alterations were observed in histology of liver and kidney by sequentially increasing both the dosage and duration of treatment. Subsequently, regular monitoring of liver and kidney function tests in patients undergoing chemotherapeutic regimen with administration of ahepato and nephroprotective agent becomes vital.

Fallopian Tube Carcinoma In-situ in Endometrial Curettage.

Primary fallopian tube carcinomas (PFTC) are rare tumors with non-specific clinical presentations. The current case was unique since the tumor was first detected on endometrial curettage and clinicoradiologically was misdiagnosed as endometrial carcinoma. A 48-year-old, post-menopausal female presented with one episode of vaginal bleeding. Endometrial curettage showed poorly differentiated carcinoma, while cervicovaginal Papanicolaou (Pap) smear was negative for malignant cells. Right sided fallopian tube carcinoma in-situ was diagnosed on histopathological examination of surgical hysterectomy with B/L salpingo-oophorectomy specimen. As observed in the current case, unusual tumor histology with broad papillary fronds lined by pleomorphic cells showing nuclear stratification and focal involvement of endometrial curettage specimen may be considered a useful pointer for tubal malignancy.

Myeloid sarcoma: A clinicopathological study with emphasis on diagnostic difficulties.

BACKGROUND: Myeloid sarcoma (MS) is a rare tumor composed of proliferation of myeloid precursors at extramedullary sites. They can arise de-novo or in association with hematological malignancies, most commonly acute myeloid leukemia. Clinically, it can masquerade as an abscess, cutaneous ulcer, or mass lesion. Morphologically, MS can mimic a variety of small round cell tumors including lymphomas and rhabdomyosarcoma. AIMS: (1) To study the clinical presentations and laboratory findings in patients with MS; (2) to revisit the histomorphological findings and the differential diagnosis of MS; (3) to evaluate the diagnostic role of immunohistochemistry (IHC) and determine the useful markers for accurate diagnosis of MS. MATERIALS AND METHODS: We reviewed cases of MS reported in our institution over a 10-year period from January 2004 to December 2013. The clinical presentations, laboratory data, and histopathological and immunohistochemical findings were studied. RESULTS: There were nine cases in our database, none of which were clinically suspected to be MS. Age ranged from 3 to 55 years, with a slight female preponderance. Cervical lymph nodes were the most common site involved. Histologically, the common finding was the presence of medium- to large-sized cells with fine granular chromatin, small nucleolus, and scant cytoplasm along with scattered eosinophil precursors. Myeloperoxidase was the most useful IHC marker. All cases were also positive for leukocyte common antigen contributing to the diagnostic confusion with lymphoma. CONCLUSION: The possibility of MS should be considered when dealing with unusual lymphoma-like neoplasms that cannot be categorized as any of the Non-Hodgkin lymphoma subtypes.

Multiple Lipomatous Hemangiopericytomas in the Foot and Ankle.

Since its first description, the diagnosis and terminology of hemangiopericytoma have been in controversy. We report perhaps the first case of multiple lipomatous hemangiopericytoma occurring in the foot and ankle region.