Anuradha Ananthamurthy

Orbital Inflammation With IgG4-Positive Plasma Cells: Manifestation of IgG4 Systemic Disease

OBJECTIVE To describe clinical, radiographic, and morphologic findings in patients with IgG4-positive cells present on orbital biopsy specimens. DESIGN Retrospective review (from January 1, 1993, through December 31, 2006) of patients with orbital biopsy specimens that excluded lymphoma; comparison of patients with and without IgG4-positive cells on immunostaining. RESULTS Of 21 patients, 11 had increased IgG4-positive cells (defined as >10 cells on biopsy). Symptoms included eyelid or periocular swelling (8 patients) or proptosis (3 patients), with bilateral involvement in 6 patients. Computed tomographic imaging displayed lacrimal gland mass in 10 patients; 6 patients had lesions in other organs. Two patients had increased serum IgG4 levels. In 10 patients without IgG4-positive cells (≤10 cells on biopsy), 6 had proptosis, 1 had eyelid swelling, 2 had eyelid mass, and 1 had diplopia, all unilateral. None had systemic symptoms. Patients with IgG4-positive cells had longer symptom duration, and their biopsy specimens showed more background fibrosis, lymphoid hyperplasia, plasma cells, and eosinophils. CONCLUSIONS The clinical appearance, high incidence of bilateral disease, association with lesions in other organs, and increased IgG4 serum levels in some patients-with an increased number of IgG4-positive cells in the biopsy specimen, which shows more background fibrosis, lymphoid hyperplasia, plasma cells, and eosinophils-indicate that these patients have an orbital manifestation of IgG4-associated systemic disease.

An unusual case of primary osteosarcoma of the rib in an adult

Primary osteosarcomas are one of the most common malignant bone tumors principally affecting the long bones in children and adolescents. An unusual case of a primary osteoblastic osteosarcoma of the rib in a 42-year-old male is presented here. The patient underwent a wide excision of the tumor and chest wall reconstruction. Although clinically unsuspected in this unusual site, the classic microscopic feature of a ramifying osteoid matrix amidst the tumor cells was diagnostic of an osteosarcoma.

Fulminant amebic colitis: a study of six cases.

BACKGROUND Amebic colitis although common, rarely presents as fulminant colitis which has a high morbidity and mortality unless treated promptly and appropriately. AIM To study the clinical, morphological features and outcome of fulminant amebic colitis (FAC). MATERIALS AND METHODS A retrospective study of six patients who underwent surgical resections from 2002-06 and were diagnosed with FAC, was carried out. The morphological features assessed included the average number of trophozoites per high-power field and the depth of invasion of trophozoites into the muscularis propria. RESULTS The study included five adults and one child who underwent surgery for fulminant colitis. Interestingly, a definite preoperative diagnosis of amebic colitis was made only in one patient and suspected in another. Intraoperatively, multiple perforations of the intestine with peritonitis were the most common findings. Gross examination typically revealed multiple ulcers with exudate and intervening normal mucosa. Microscopically, ulceration and myonecrosis with trophozoites within the exudate were seen in all cases. Trophozoites invading the muscularis propria were seen in five cases. Of the cases that showed myoinvasion by trophozoites, two patients expired within two weeks of surgery. One of the patients who expired also showed co-infection with Actinomyces. CONCLUSION FAC is an uncommon outcome in amebic colitis with a high mortality requiring prompt surgical intervention.

Atypical extragonadal germ cell tumors

Aim: To review the experience with the diagnosis and management of extragonadal germ cell tumors (GCT) with a subset analysis of those with atypical features. Materials and Methods: A retrospective chart review of patients of extragonadal germ cell tumors between 2000 and 2010 was carried out. Results: Fifteen children aged 7 days to 15 years (median, 1.5 years) were included. Three had an antenatal diagnosis (one sacrococcygeal, one retrobulbar, one retroperitoneal tumor) and were operated in the neonatal period. The locations were distributed between the retrobulbar area (1), anterior neck-thyroid gland (1), mediastinum (4), abdominothoracic extending through the esophageal hiatus (1), retroperitoneal (4) and sacrococcygeal (4). On histological examination, five harbored immature elements while two were malignant; the latter children received postexcision adjuvant chemotherapy. There was no mortality. At a median follow-up of 4.5 years (6 months to 8 years), 14/15 have had an event-free survival. One immature mediastinal teratoma that recurred locally 7.5 years after the initial operation was excised and adjuvant chemotherapy instituted. Conclusions: Extragonadal GCTs in children are uncommon and occasionally present with atypical clinical, radiological and histological features resulting in diagnostic and therapeutic dilemmas.

Clinicopathological attributes of T-lymphoblastic lymphoma seen in a tertiary care centre

Background and Aims: T-lymphoblastic lymphoma (T-LBL) is a type of non-Hodgkin lymphoma (NHL), the cell of origin being the precursor T cell. This study was undertaken to describe the distribution, clinical presentation, morphological spectrum, immunohistochemical profile, and outcomes in patients with LBL presenting to our institution which is a tertiary care center. Methods: A total of 41 cases of T-LBL diagnosed during a 7-year period were included in this study. These patients were stratified into T-LBL cases and T-LBL/acute lymphoblastic leukemia cases, the latter defined as those with a lymphomatous mass and more than 25% blasts in the bone marrow. Medical records were reviewed for clinical, laboratory data, imaging findings, treatment, and follow-up. The histopathology and immunohistochemistry slides were reviewed. Results: T-LBL constituted 8.4% of all NHL seen in the period. This lymphoma is most common in childhood and adolescence. Mediastinal compression and pleural effusion are very common in patients with T-LBL (65% and 40%, respectively). The morphology consists of small-to-medium sized blasts that typically are positive for CD3, CD99, and TdT. T-LBL is an aggressive disease; relapse and progression being markers of poor outcome. Conclusion: This study is a comprehensive account of T-LBL from a tertiary care center in South India which describes the distribution, clinicopathological attributes and outcome in patients with this aggressive form of NHL.

HIV-associated lymphomas: A clinicopathological study from India

Background: Although there is a high burden of HIV-positive cases in India, there is very little published data on the prevalence and pathological profile of malignancies occurring in these patients. Aims: The current study was undertaken to analyze the clinicopathological profile of HIV-positive patients with Hodgkin lymphoma (HL) and non-Hodgkin lymphoma (NHL). Materials and Methods: This was a retrospective study over a 6-year period consisting of histopathology slide and clinical chart review of HIV-positive patients with lymphomas. Results: Of a total of 3470 HIV-positive patients during the 6-year period of study, the number of HIV patients diagnosed with lymphomas was 26. The mean duration between HIV seropositivity and development of lymphoma was 12 months. Seven patients were diagnosed as HIV positive during the course of investigations after a lymphoma diagnosis. The male to female ratio was 5.5:1 and the mean CD4 count, 171/mm3. Among the 26 cases, 3 were diagnosed with classical HL and the rest with NHL. B-cell lymphomas predominated with 19 cases. Among the NHLs, plasmablastic lymphoma and diffuse large B-cell lymphoma were the common subtypes with no cases of either Burkitt Lymphoma or primary CNS lymphoma. The median duration of follow-up was 1 year. Ten cases died during the course of treatment, 11 cases achieved complete remission. Conclusions: This is one of the few studies from India documenting the clinicopathological features of HIV-associated lymphomas. It is interesting that in many cases, HIV positivity was detected during the course of investigations after a lymphoma diagnosis.