Ranajoy Ghosh

Cytomorphological and Morphometric Analysis of 22 Cases of Rosai-Dorfman Disease: A Large Series from a Tertiary Care Centre

Background: Rosai-Dorfman disease (RDD) is a rare benign idiopathic histiocytic proliferation. Most commonly reported cases are lymph nodal. Only 30-40% of cases occur in extranodal sites. Although the morphological features of RDD are well documented, there is limited information about the morphometric variations in the histiocytes of this entity. Method: Twenty-two cases of RDD diagnosed on fine-needle aspiration cytology (FNAC) were retrieved from the archives. Both Papanicolaou- and May-Grünwald-Giemsa-stained slides were available for evaluation in all cases. Nuclear area, diameter and histiocyte size were measured taking reactive histiocytes as controls. Results: Among the 22 patients (male/female ratio 3:2; age range 5-55 years, mean 26 years), 3 cases were extranodal and 19 cases were nodal. The nodal sites included cervical, axillary, inguinal and submandibular lymph nodes. The extranodal sites were the retroperitoneum, mediastinum and skin. The most common clinical presentation was enlarged lymph nodes. Cytological features included numerous large benign histiocytes with emperipolesis. All the morphometric parameters were significantly (p < 0.01) higher in RDD histiocytes than in histiocytes in the reactive lymph node. Conclusions: In view of the rarity of the disease and the variable clinical presentation in RDD, accurate diagnosis is necessary. This is the first study to document the morphometric parameters of RDD histiocytes and their comparison with histiocytes in the reactive lymph node.

Primary Renal Rhabdomyosarcoma: An Unusual Bone Metastasizing Tumor of Kidney

ABSTRACT Rhabdomyosarcoma (RMS) is one of the common malignant tumors in the pediatric age group. There is only a single case report of primary renal alveolar RMS. Fine needle aspiration (FNA) findings of primary renal RMS has not been reported so far. Hence we present an unusual case of primary alveolar RMS of the kidney. An 11 year old boy presented with an abdominal mass. On FNA a diagnosis of undifferentiated sarcoma and anaplastic Wilms tumor were considered. The tumor was resected and showed histopathological features of alveolar rhabdomyosarcoma. He developed multiple bony metastases and succumbed to the illness despite aggressive chemotherapy. RMS of the kidney should be considered in the differential diagnosis of children with a renal mass, and may have an aggressive clinical course with bone metastases.

Lipoblastoma and lipoblastomatosis: A clinicopathological study of six cases

Lipoblastoma and lipoblastomatosis are benign adipocytic tumors seen in children less than 3 years of age. Awareness amongst surgeons of this fast growing lipomatous tumor in young children is important to avoid mutilating surgery and chemotherapy. Histological diagnosis of lipoblastoma is sometimes difficult because of close resemblance with myxoid, round cell, and well-differentiated liposarcoma. We came across a series of six cases of lipoblastoma/lipoblastomatosis, over the last 10 years (2002-2012). Of these, we had three cases of lipoblastomatosis, one of which recurred after 2 months. All others were resected completely and successfully.

Recurrent sebaceous carcinoma of the scalp in a young male treated with adjuvant radiotherapy

Sebaceous carcinoma is a rare malignancy of the skin appendages. It tends to occur in elderly patients. Orbital region is the most commonly involved site seen in 75% of cases. The involvement of extra-orbital sites is infrequent. Herein, a case of extra-ocular sebaceous carcinoma arising in the scalp of a 20-year-old man is described.The patient developed tumor relapse after excision biopsy. He was treated with wide local excision of the tumor. However, the patient developed local recurrence after an interval of four months for which he again underwent wide local excision. He did not manifest any regional or distant metastases. In view of the locally aggressive tumor, he received adjuvant radiation therapy. The patient was successfully treated with no evidence of any local recurrence seen after a follow-up period of one year. The timely recognition of sebaceous carcinoma is imperative so as to execute the primary treatment i.e., wide local excision. Adjuvant radiotherapy may be considered to improve the clinical outcome for recurrent tumors.

Myxoid adrenal cortical adenoma in an infant: An unusual morphology.

Myxoid adrenal cortical neoplasms are rare. To the best of our knowledge, no such case has been reported in pediatric or infantile age group till now. Here we report a case of non-functional myxoid adrenocortical adenoma (ACA) in a 7-month-old girl, who presented with a large mass in the abdomen. Microscopically, the tumor was composed of alveolar clusters of cells with focal pseudoglandular architecture in a background of abundant alcian blue positive myxoid matrix. Compressed rim of adrenal tissue was identified at periphery. The patient was put on a close follow-up in view of scarce literature on the subject. She has been doing fine without any recurrences. Myxoid adrenal cortical tumors expand the differential diagnoses of a myxoid neoplasm in retroperitoneum.

Potter's sequence: a story of the rare, rarer and the rarest.

Potters syndrome is a rare condition affecting one in 2000-5000. We present here two autopsy cases of Potters syndrome, with the rare finding of discoid adrenals and the even rarer finding of in situ neuroblastoma in one of the cases.

INI Expressing Epithelioid Sarcoma with Osteoclastic Giant Cells in a Child: A Case Report with Summary of Prior Published Cases

ABSTRACT Background: Epithelioid sarcoma is a heterogeneous tumor with 2 subtypes, classic and proximal. The proximal variant is more aggressive and occurs in proximal location in young adults. Case Report: We present a proximal epithelioid sarcoma in the leg of an 8 year old girl with rhabdoid morphology and scattered osteoclastic giant cells. Nuclear INI-1 was retained. Despite wide local excision, local recurrence occurred at 8 months. Following re-excision, she developed a chest wall metastasis after 9 months. Conclusion: Epithelioid sarcoma, proximal type with osteoclastic giant cells in the pediatric age group has not been reported previously and should be considered in the differential diagnoses of tumors with epithelioid cell morphology and scattered osteoclastic giant cells. Retained INI expression helped to differentiate this tumor from malignant rhabdoid tumor.

Expression of COX-2 and p53 in juvenile polyposis coli and its correlation with adenomatous changes

INTRODUCTION Gastrointestinal polyps commonly affect the pediatric population. The commoner variety amongst these is the solitary rectal polyp. Juvenile polyposis coli (JPC) is rare, characterized by multiple polyps occurring throughout the gut. AIM The role of cyclooxygenase-2 (COX-2) has been implicated in gastrointestinal tumorigenesis. We aimed to look at the clinicopathological spectrum of solitary vs juvenile polyposis and compare their differences in expression of COX-2 and p53. MATERIALS AND METHODS We studied 38 polyps from eight cases of JPC, collected over the past 10 years along with 40 solitary rectal polyps (SRP). RESULTS The size of polyps was significantly more in cases of JPC compared to SRP. Adenomatous change was observed significantly more often in JPC. COX-2 expression was also significantly higher in the JPC group compared to SRPs. All cases of JPC polyps with adenomatous change showed strong COX-2 expression. There was no significant difference in expression of p53 in the JPC and SRP groups. CONCLUSION We observed significantly higher COX-2 expression in JPC. Establishment of the role of COX-2 in JPC will help us formulate chemopreventive therapies as an adjunct to its surgical management.