Kaushik Majumdar

Inflammatory myofibroblastic tumor appendix with concomitant mucosal dysplasia, simulating pseudomyxoma on preoperative aspiration cytology.

Inflammatory myofibroblastic tumor (IMT) has been described as a pseudosarcomatous proliferation of spindled myofibroblasts admixed with lymphoplasmacytic cells. The various terminologies like inflammatory pseudotumor, plasma cell granuloma, and inflammatory myofibrohistiocytic proliferation, used to describe this entity, highlight the controversial etiopathogenesis of this relatively indolent neoplasm. IMT has now been described in different anatomic locations. However, cases occurring in the gastrointestinal tract are rare with very few cases described in the appendix. We present a case of inflammatory myofibroblastic tumor appendix with mucosal dysplasia in a 41-year-old male, presenting with abdominal pain and lump in the right iliac fossa. Aspiration cytology yielded few atypical epithelial cells and spindle cells in a mucinous background, suggesting the possibility of pseudomyxoma peritonei. Awareness of IMT appendix with rare presence of mucosal dysplasia may help in preventing overzealous resection, especially in situations that on preoperative evaluation may suggest malignancy.

Multicentric Extranodal Rosai Dorfman Disease – A Cytological Diagnosis, with Histological Corroboration

Background: Rosai Dorfman disease (RDD) typically presents with massive bilateral cervical lymphadenopathy, a viral-like prodrome, elevated erythrocyte sedimentation rate, and polyclonal hypergammaglobulinemia. Other lymph nodes may be less commonly involved. Extranodal RDD is quite rare, and orbital disease accounts for only 10% of the extranodal sites of involvement. Multicentric disease has also been described, which is usually accompanied by lymphadenopathy either initially or later in the disease course. Case: We report an extremely rare extranodal multicentric disease in a diabetic patient, presenting with bilateral orbital involvement, causing ocular motility restriction, which was diagnosed on aspiration cytology of the orbital mass. This was followed in quick succession by new mass lesions in the lower back and infratemporal fossa. On extensive work-up, no lymphadenopathy was detected. The patient responded well to surgical debulking of the orbital lesions and systemic steroids. Conclusion: Fine needle aspiration cytology can be effectively applied for early diagnosis of multicentric extranodal RDD. Surgical debulking in such cases may be supplemented by systemic steroids.

Co-occurrence of cytomegalovirus-induced vanishing bile duct syndrome with papillary stenosis in HIV infection

Jaundice in patients with AIDS can be a result of diverse conditions ranging from opportunistic infections to drug‐related hepatotoxicity. With the advent of antiretroviral therapy (ART), the prevalence of AIDS cholangiopathy as a cause of jaundice has decreased; on the other hand, ART‐related hepatotoxicity has become one of the commonest causes of jaundice in these patients. AIDS cholangiopathy is a rare condition of extrahepatic biliary obstruction in patients with advanced HIV infection, usually due to opportunistic infections. Vanishing bile duct syndrome (VBDS) is an acquired disorder characterized by progressive destruction and loss of interlobular bile ducts causing intrahepatic cholestasis. Herein, we report co‐occurrence of fatal cytomegalovirus (CMV)‐induced VBDS along with papillary stenosis, as a component of AIDS cholangiopathy, which to the best of our knowledge has not been documented earlier. This is perhaps the third case of VBDS in a patient with AIDS, and the second in association with CMV infection. VBDS in AIDS has a poor outcome, and liver transplantation may be considered only in a suitable candidate.

Gliosarcoma with osseous tissue: an occasional metaplastic component

Glioosarcomas represent a rare entity of intrinsic CNS neoplasms consisting of glial and sarcomatous elements; they account for 2xa0% of glioblastomas. There have been few reports of metaplastic osseous transformation in gliosarcomas. Here we report a rare case of gliosarcoma with metaplastic osteoid in a 57-year-old male patient. Magnetic resonance imaging revealed a large solid cystic heterogeneous mass lesion in the left temporal lobe with peri-tumoral oedema and areas of calcification. Histology revealed a grade IV tumour with neoplastic glial and mesenchymal components with myxoid change and areas of necrosis. The sarcomatous areas contained regions of benign osteoid formation. The histogenesis of the osseous element in gliosarcomas is not well understood, and could be a result of a metaplastic change or divergent differentiation from a common progenitor cell. The osseous tissue may be in the form of newly formed osteoid, benign bone tissue formation with lacunae and osteoblastic rimming, or unequivocally malignant tissue resembling an osteosarcoma.

Sphenoid wing lymphoplasmacyte-rich meningioma with occasional emperipolesis closely simulating an intracranial Rosai-Dorfman disease: a diagnostic dilemma.

In lymphoplasmacyte-rich meningioma (LRM) meningothelial whorls are overshadowed by exuberant infiltration by lymphocytes, plasma cells and few histiocytes. Hence, lesions with lymphoplasmacytic proliferation form the histological differentials. We describe the, to the best of our knowledge, first case of LRM with occasiona emperipolesis, creating a diagnostic dilemma with Rosai-Dorfman disease (RDD), around the region of sphenoid wing. LRM was favored due to the presence of epithelial membrane antigen (EMA) and vimentin positive meningothelial whorls, forming approximately 10% of the tumor tissue. Documentation of such cases may help to understand the importance of inflammatory cells and meningothelial whorls, as a manifestation of host response at the leptomeninges.

Cytological findings of odontogenic myxofibroma: A diagnostic dilemma.

Odontogenic myxofibroma represents a rare slow‐growing benign neoplasm, which usually occurs in the second and third decades of life and rarely in children or adults over 50 years of age. Myxomas in general represent from 2.3% to 17.7% of all odontogenic tumors, and myxofibromas represent a small number of all myxomas. Limited evidence is present in literature regarding the cytological diagnosis of odontogenic myxoma/myxofibroma. We hereby report the cytomorphological features of a histologically confirmed case of odontogenic myxofibroma and the pitfalls of the cytological diagnosis. A painless jaw swelling in a young boy was aspirated. Scanty mucoid material was obtained. Cytology Smears were moderately cellular and showed a population comprising predominantly of singly scattered plump to fusiform cells with bipolar cytoplasmic processes showing mild to moderate atypia embedded within dense myxoid matrix and another population of cells arranged in clusters. Case was interpreted as low grade mesenchymal tumor. Subsequent biopsy confirmed it as odontogenic myxofibroma arising in a odontogenic keratocyst. Precise interpretation of intraosseous jaw lesions FNAC may not always be possible, but an attempt should be made to broadly classify the lesion as an inflammatory lesion, cystic lesion, giant cell lesion, fibro‐osseous lesion or as an odontogenic tumor. If dual population of odontogenic epithelium and mesenchymal cells embedded in myxoid matrix are identified in such aspirates, a possibility of myxoid odontogenic tumor may be suggested. Triple correlation of cytological, clinical and radiological findings can guide the surgeon for taking appropriate therapeutic decisions. Diagn. Cytopathol. 2016;44:329–333.

Isolated Lichen Planus of Lower Lip: A Case Report

Lichen planus is an idiopathic inflammatory condition, which may involve mucosa of the oral cavity, gastrointestinal tract, larynx or the cutaneous surface either in isolation or in combinations. Mucosal lichen planus is more common than the cutaneous variant. Isolated lip involvement is very rare and should be differentiated from other similar leukoplakic lesions. We are reporting a rare case of oral lichen planus in an elderly male that was exclusively localised to the lower lip.

Role of intraoperative squash smear cytology as a diagnostic modality in lipoma of quadrigeminal cistern

Quadrigeminal lipoma is a rare tumor that has been categorized as developmental malformation rather than a hamartoma or true neoplasm, due to its origin from abnormal persistence and mal-differentiation of meninx primitiva during the development of the subarachnoid cisterns. Reported admixture of adipose tissue with heterotopic elements also supports a developmental origin. Quadrigeminal lipomas are frequently asymptomatic and detected incidentally. Though a favorable clinical course is usually expected, recurrences may occur due to partial removal of lesions in close relation to vital structure. We describe the role of intraoperative squash smear cytology as a diagnostic aid in quadrigeminal cistern lipoma and an alternative to frozen sections that are technically difficult to obtain due to presence of lobules of fibro-adipose tissue. With radiological correlation, squash cytology can be an economical method for intraoperative diagnosis, pending subsequent histopathological confirmation.

Negative shadows conveying a positive note.

A 45-year-old male presented with a nodule below the right elbow, measuring 1 cm in diameter. The nodule was present since 8 months, and the overlying skin was excoriated. The patient gave history of decreased sensations over the lateral side of both feet. On physical examination he had multiple thickened nerves. With clinical differential diagnosis of leprosy (Hansen’s disease), sarcoidosis and deep fungal infection patient underwent fine needle aspiration cytology (FNAC). Aspiration of the nodule was performed using 23-gauge needle mounted on a Cameco’s handle. The material obtained was fluid and blood mixed. Air-dried smears were stained with WrightGiemsa and few slides were kept reserved for spatial stains. Smears were moderately cellular and showed abundant foamy macrophages, neutrophils, and lymphocytes. The cytoplasm of histiocytes showed aggregates of negative images of bacilli (Figs. 1a and b). Fite stain was performed on the reserved air-dried slides which showed globi of Lepra bacilli in the cytoplasm of histiocytes along with scattered bacilli in the smear background (Fig. 2). Subsequently, a punch biopsy from skin over back was performed and submitted for histopathological examination. Sections showed atrophic epidermis with a subepidermal clear zone. The underlying dermis showed spindle shaped histiocytes arranged in storiform pattern (Figs. 3 and 4). Few admixed lymphocytes were also seen. Fite stain for Lepra bacilli was strongly positive (Bacteriological Index 61) (Fig. 5). Final histopathological diagnosis of histoid leprosy was given. Leprosy is a chronic infectious disease caused by Mycobacterium leprae chiefly involving the skin and peripheral nerves. The histopathological findings in leprosy are related to the immunological status of the patient. Ridley and Jopling proposed a five group histological classification reflecting the immunological spectrum: Tuberculoid (TT), Borderline Tuberculoid (BT), midborderline (BB), Borderline Lepromatous (BL), and Lepromatous (LL). Later on they developed clinical and bacteriological finding in each of these groups. Clinicians have also adopted the Ridley Jopling nomenclature for classifying leprosy on clinical grounds. Histoid leprosy is a form of lepromatous leprosy and exhibits characteristic clinical and histological features. It was first described by Wade in 1963. Patients present with firm, reddish, or skin-colored, dome-shaped or oval papules and/or nodules, regular in contour with stretched overlying skin. The lesions grow in an expansile fashion on apparently normal skin. They usually occur over the lower back, buttocks, face, extremities, and bony prominences and may appear de novo or after inadequate treatment. The overlying epidermis is atrophic and in most cases, a free subepidermal zone (Grenz) is present. On histology sections lesions show numerous, thin, spindle shaped histiocytes forming interlacing bands and whorls along with foamy macrophages. Acid fast stain shows a bunch of acid fast bacilli within the histiocytes and also extracellularly. Department of Pathology and Laboratory Medicine, All India Institute of Medical Sciences, Bhopal, India Department of Pathology, GB Pant Hospital, New Delhi, India *Correspondence to: Dr. Deepti Joshi MBBS, MD, Associate Professor, Department of Pathology and Laboratory Medicine, All India Institute of Medical Sciences, Bhopal, India. E-mail: deepti.patho@ aiimsbhopal.edu.in Received 27 August 2015; Revised 21 January 2016; Accepted 26 January 2016 DOI: 10.1002/dc.23455 Published online 16 February 2016 in Wiley Online Library (wileyonlinelibrary.com).

Cerebellar metastases of recurrent phyllodes tumor breast; a rare phenomenon reflecting the unpredictable outcome.

Carcinomas of lung, breast, colon, kidney, and malignant melanomas are the most common malignancies that metastasize to the central nervous system (CNS). Phyllodes tumor is a rare fibroepithelial tumor of the breast, often having unpredictable recurrences, with increasing histological grade and distant metastasis. Malignant forms exist, which may develop distant metastases usually to the lung, pleura, bone, and liver. CNS metastasis of phyllodes tumor is rare and associated with a poor prognosis, with resistance to chemotherapy and radiation. We present a rare case of cerebellar metastasis in recurrent phyllodes tumor breast with subsequent rapid downhill course.