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.

Clinical and pathological characteristics of blastoid mantle cell lymphoma: a single institution experience.

Background: Blastoid mantle cell lymphoma (B-MCL) is a rare aggressive lymphoma. It is characterized by blastoid morphology with high proliferation and inconsistent immunohistochemistry (IHC), making it a diagnostic challenge for the pathologist. Methods: This is a retrospective analytical cohort study. We reviewed biopsy confirmed cases of B-MCL diagnosed over a period of 10 years (January 2012 to December 2022). The clinical presentation, histopathological and IHC findings, treatment received, and survival outcomes were studied. Randomly selected cases of classic MCL (n=12), diagnosed during the same period served as controls. Results: A total of 12 cases were studied. Four cases were transformed from previously diagnosed MCL; 8 cases arose de novo. Mean age was 61.17 years and the male: female ratio was 5:1. Half of the cases showed extra nodal extension and 81.8% had bone marrow involvement. Gastrointestinal tract was the most common site of extra nodal involvement. Histopathological examination showed diffuse involvement of the lymph node with medium sized cells. On immunohistochemistry, one of the cases showed loss of CD5 expression while the other had aberrant CD10 expression. Mean Ki-67 index was 58.09% in the cases and 16.33% in controls and was statistically significant ( p=0.005). The median overall survival (OS) for cases was 2 years vs 8 years in controls. The p53 over expression (>30% nuclear positivity) was seen in 66.6% cases (4/6). Conclusion: There are several factors that contribute to the aggressiveness of B-MCL, and new treatment approaches might be required to improve patient outcomes.

Benign scalp lesion: An unusual presentation of B cell- lymphoblastic lymphoma - A case report.

This case report highlights the occurrence of B- cell lymphoblastic lymphoma (B-LBL) as a solitary cutaneous lesion without an existing systemic involvement and should be kept in the differentials while dealing with cases presenting with a similar clinical picture. We report the case of a 13-year-old girl who presented with a painful, progressively enlarging swelling in right zygomatico-temporal region, clinically simulated a deep fungal infection/granulomatous lesion and turned out to be a case of B-LBL without any systemic involvement on further work up. This case is being reported to emphasize that B-LBL should be considered as a differential for an otherwise benign appearing persistent lesion in the head and neck region.

"Giant cell fibroma of buccal mucosa -an unusual lesion of unusual size": A case report.

Giant cell fibroma is a benign oral fibrous tumour. The clinical appearance of majority of non-neoplastic fibrous growths is similar, but unique histopathological features of giant cell fibroma aid in its final diagnosis. It usually manifests as an asymptomatic, sessile or pedunculated mass usually less than 1 cm in diameter. In this case report, we highlight a case of giant cell fibroma in a 58-year-old male patient, which had an unusual size with associated pain. Although giant cell fibromas are benign lesions, it is important for dentists to be aware of this lesion based on its frequency of occurrence and need for its accurate diagnosis.

Sertoli-Leydig cell ovarian tumour: a rare cause of virilisation and androgenic alopecia.

Sertoli-Leydig cell tumours (SLCTs) represent a rare cause of hyperandrogenic state. SLCTs are sex cord ovarian neoplasms, accounting for <0.2% of all ovarian tumours. Most of the sex cord-stromal tumours have a benign clinical course, with 10%-20% of them at risk of aggressive course. We report a case of a woman in her 30s who presented with androgenic alopecia, virilisation and secondary amenorrhoea. The evaluation revealed an extremely high testosterone level. Imaging for the localisation of source of excess testosterone with contrast-enhanced CT of the abdomen revealed a right ovarian mass. Hence, a diagnosis of testosterone-secreting ovarian tumour was considered. The patient underwent right salphingo-oophorectomy, and histopathology was reported as Sertoli cell tumour. Postoperatively, there was normalisation of serum testosterone levels with decrease in virilisation and resumption of spontaneous menstrual cycles. The patient conceived spontaneously after 2 months of surgery.

Concomitant malignancies in the neck: follicular dendritic cell sarcoma; a rare tumour presenting as a right-sided neck nodal mass and papillary carcinoma thyroid.

Follicular dendritic cell sarcoma is a rare low-grade sarcoma of mesenchymal origin. It involves the lymph nodes more commonly and rarely extranodal sites. The most common lymph node is cervical and usually presents as a painless asymptomatic mass. More often, it is a misdiagnosis, and there is a delay in treatment. It is rarely associated with Castleman disease, myasthenia gravis. Diagnosis of this condition is by histopathology and immunochemistry. Surgery is the primary modality of treatment, and adjuvant therapy has been tried with no definite trials due to the rarity of the disease. Here, we report a case of concomitant follicular dendritic sarcoma of the right cervical lymph node and papillary carcinoma of the thyroid managed in our institute. There was a line of investigations approaching towards a diagnosis, and she underwent total thyroidectomy and right modified radical neck dissection.

Oro-facial aspects of leprosy: report of two cases with literature review.

Leprosy is a chronic infectious disease affecting primarily the skin, peripheral nerves, respiratory system and the eyes. Leprosy induces various types of clinical presentation affecting the patient's immune response. Cell-mediated immunity is considered to be the crucial defence against the disease and the magnitude of this immunity defines the extent of the disease. The article presents two case reports of manifestations of leprosy in the oro-facial region, with a brief review of various other important oro-facial manifestations of leprosy. The first report deals with granulomatous nodules in the palate while the second report presents bilateral facial palsy in leprosy patients. Both the reports gain importance due to rare oral manifestation in a borderline leprosy patient in the first case, while the second case presents a rare bilateral Bell's sign. The role of the dental profession and especially the Oral Medicine specialist is of great importance in early diagnosis of oral lesions.

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.

Embryonal Rhabdomyosarcoma - A Mimicker of Squamosal Otitis Media.

INTRODUCTION: Rhabdomyosarcoma is the most frequently occurring intrusive soft tissue sarcoma in the pediatric age group. Orbit is the most common location for a pediatric rhabdomyosarcoma, but it can occur in the oral cavity, pharynx, face and neck in the descending order of incidence. Rhabdomyosarcoma in the ear is extremely rare. CASE REPORT: A 5-year-old girl presented to the outpatient department of our tertiary care hospital with complaints of foul smelling, non-blood stained right ear discharge of one-month duration and deviation of angle of mouth to the left side of acute onset. Investigations revealed a diagnosis of embryonal rhabdomyosarcoma. Multimodal therapy was carried out, and the child was rendered disease-free after two years. CONCLUSION: Embryonal rhabdomyosarcoma of the head and neck mimics chronic otitis media. Early diagnosis is essential to deliver prompt treatment and prevent locoregional spread and metastasis.

Deep Convolution Neural Network for Malignancy Detection and Classification in Microscopic Uterine Cervix Cell Images.

OBJECTIVE: Automated Pap smear cervical screening is one of the most effective imaging based cancer detection
tools used for categorizing cervical cell images as normal and abnormal. Traditional classification methods depend on
hand-engineered features and show limitations in large, diverse datasets. Effective feature extraction requires an efficient
image preprocessing and segmentation, which remains prominent challenge in the field of Pathology. In this paper, a
deep learning concept is used for cell image classification in large datasets. METHODS: This relatively proposed novel
method, combines abstract and complicated representations of data acquired in a hierarchical architecture. Convolution
Neural Network (CNN) learns meaningful kernels that simulate the extraction of visual features such as edges, size,
shape and colors in image classification. A deep prediction model is built using such a CNN network to classify the
various grades of cancer: normal, mild, moderate, severe and carcinoma. It is an effective computational model which
uses multiple processing layers to learn complex features. A large dataset is prepared for this study by systematically
augmenting the images in Herlev dataset. RESULT: Among the three sets considered for the study, the first set of single
cell enhanced original images achieved an accuracy of 94.1% for 5 class, 96.2% for 4 class, 94.8% for 3 class and
95.7% for 2 class problems. The second set includes contour extracted images showed an accuracy of 92.14%, 92.9%,
94.7% and 89.9% for 5, 4, 3 and 2 class problems. The third set of binary images showed 85.07% for 5 class, 84%
for 4 class, 92.07% for 3 class and highest accuracy of 99.97% for 2 class problems. CONCLUSION: The experimental
results of the proposed model showed an effective classification of different grades of cancer in cervical cell images,
exhibiting the extensive potential of deep learning in Pap smear cell image classification.

Nodular Fasciitis - Fine Needle Aspiration Cytology Diagnosis and Its Pitfalls, with Review of Literature.

BACKGROUND AND OBJECTIVE: Nodular fasciitis (NF) is a self-limiting, transient neo- plasm composed of fibroblasts and myofibroblasts. Since it regresses spontaneously, diagnosis by fine needle aspiration (FNA) cytology plays a major role in its management. METHODS: We present a series of 8 cases with either FNA or biopsy diagnosis ofNF, and study the major cytological features with a review of literature on diagnostic criteria and its pitfalls. RESULTS AND CONCLUSION: The 8 cases occurred in patients between the age of 14 to 72 years, with equal sex distribution. FNA diagnosis concurred in 4 cases. Causes of wrong diagnosis included lack of clinical information and paucicellular smear. FNA cytology is an important tool in the diagnosis of nodular fasciitis, in appropriate clinico-radiologicalsetting.

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.

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.

Rhinofacialentomophthoromycosis.

Entomopthoromycosis is a rare subcutaneous fungal infection caused by onidiobolus coronatus affecting mainly the upper respiratory mucosa in immunocompetent people.The manifestations of this disease masquerades other clinical entities.Hence, high index of suspicion is required for prompt diagnosis.Histopathological examination and culture are the gold standard diagnostic tools, however no standard treatment protocols ha been mentioned in literature.We present a case of rhinofacial entomopthoromycosis in a yearold ma with a leftsided rhinofacial swelling to highlight the presence of this unusual fungal infection and its treatment.

Cytomorphologic Diversity of Papillary Thyroid Carcinoma.

INTRODUCTION: Cytologic features of papillary thyroid carcinoma (PTC) have been extensively documented in literature. However, PTC variants can prove to be diagnostically challenging on fine needle aspiration cytology (FNAC). AIMS: To study the FNAC features of PTC and its variants and explore the causes for misdiagnosis. MATERIALS AND METHODS: This is a retrospective study. All cases of histopathologically (HP) confirmed cases of PTC during a 2-year period (January 2012 to December 2013) with presurgical FNAC were included. The cytologic findings and FNAC diagnosis of each case were documented and compared with the HP report. The misdiagnosed cases were reviewed to look for any cytological clues and reasons for misdiagnosis. RESULTS: A total of 58 cases were included. The overall diagnostic accuracy was 55.6% which improved to 64.8% on including suspicious for PTC cases. Follicular variant was the most misdiagnosed variant; 41.2% of the cases were called follicular neoplasm. Oncocytic variant showed cells with abundant eosinophilic cytoplasm along with bizarre giant cells. Warthin tumor-like variant showed cells with moderate eosinophilic cytoplasm with close apposition of lymphocytes in a background of reactive lymphocytes and lymphoid tangles. Cystic variant was paucicellular. Columnar cell variant showed tall columnar cells with nuclear stratification. Cribriform-morular variant showed syncytial sheets of cells and hyaline globules. CONCLUSIONS: PTC variants have distinct cytomorphological features. In some variants (follicular, columnar cell), nuclear grooves and inclusions may not be apparent, contributing to the diagnostic confusion. Benign nodule adjacent to the tumor can dominate the FNAC smear and lead to misdiagnosis.

Cystic Sertoli-Leydig Cell Tumour in a Postmenopausal Woman with Absent Virilising Symptoms: A Diagnostic Challenge.

A 60-year-old postmenopausal woman, presented with a left ovarian cystic mass with mildly elevated CA125 levels. An intraoperative frozen section showed oedematous ovarian stroma with interspersed large aggregates of spindle shaped stromal cells. Subsequently, the excised specimen was reported as Sertoli-Leydig Cell Tumour (SLCT) of intermediate differentiation. The leydig cells were identified in the imprint smears, but were misinterpreted as luteinized cells. The lack of tubular differentiated cells in frozen section had contributed to the misdiagnosis. Immunohistochemistry (IHC) played an important diagnostic role in the absence of clinical suspicion and lack of virilising features that are classically described in association with SLCTs. This case is unusual, as the tumour was seen in a postmenopausal woman in the absence of virilising symptoms. The cytomorphological features, IHC findings and the reasons for misdiagnosis are discussed in this case report.

Transient Thyrotoxicosis in Molar Pregnancy.

Molar pregnancy is one of the components of a broader spectrum of diseases known as Gestational Trophoblastic Disease (GTD), presenting with amenorrhoea and irregular bleeding which may be rarely associated with passage of vesicles per vagina. However, it can rarely be associated with hyperthyroidism, which may be associated with clinical features of hyperthyroidism. The following is a report of a 20-year-old woman who presented with amenorrhea followed by irregular bleeding per vagina, thyromegaly and abnormal levels of thyroid hormones. Transvaginal ultrasound revealed features consistent with molar pregnancy. A suction evacuation was done following which serum levels of ß-hCG reduced and the levels of thyroid hormones also reduced. On follow up, six weeks later, ß-hCG and thyroid hormones were within normal limits. The case and relevant literature are presented here.

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.

Ulceroproliferative growth on the heel: epithelioma cuniculatum.

Verrucous papules and plaques on the plantar surfaces should not be assumed to be mere warts, especially if the history is unusual. We present a patient with an ulceroproliferative growth on the heel which was found to represent the epithelioma cuniculatum form of squamous cell carcinoma.