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Breast hamartomas are rare benign masses composed of glandular, adipose and fibrous tissue. While it is not uncommon to see ductal hyperplasia, apocrine metaplasia or adenosis in hamartomas, rare case reports of coexistent lobular or ductal intraepithelial neoplasms and in situ carcinomas have been reported in these lesions. Imaging with ultrasound and mammography was undertaken for a patient who presented with complaints of right breast pain. Right breast malignancy was detected on mammography, and guided biopsy using USG was undertaken from the same lesion. Triple-positive right breast malignancy was detected. This case highlighted the rare occurrence of a malignancy coexisting with a fairly large breast hamartoma.

This retrospective analysis aimed to evaluate the clinicopathological characteristics of primary mucinous tumors of the ovary (MOT) treated at a tertiary care center. Demographic, clinicopathological, treatment and follow-up details of all patients with primary MOT treated from June 2015 till August 2019 were extracted from institutional cancer registry and Electronic Medical Records, after obtaining necessary clearance from institutional ethical committee, and institutional review board. The details obtained were tabulated and analyzed. Thirty-one patients were identified to have primary MOT: 20 cystadenomas, 10 carcinomas (mOC) and one borderline (mBOT). MOT was frequently noted between the fourth to sixth decades. Abdominal distension was the most common symptom at presentation. In total, 70% of cystadenomas were less than 15 cm, while 90% of mOC were 15 cm and above. In total, 95% cystadenomas were unilateral, while all cases of mBOT and mOC were unilateral. mOC showed elevated CA 125 in 42.86% cases, elevated CEA in 42.86% cases and elevated CA 19–9 in 75% cases. Imaging showed 30% of mOC as benign cysts and 42.1% of cystadenomas as complex cyst. Frozen section correctly diagnosed cystadenomas and mBOT, and two mOC were underdiagnosed as borderline. All had upfront surgery. Of cystadenomas, 11 underwent salpingoovariotomy, and nine had hysterectomy and salpingoovariotomy. All the mOC and mBOT underwent staging laparotomy. Three cases of mOC had a fertility preserving approach. No cases had positive lymph nodes, and appendix showed normal histology. In total, 40% were stage 1a, 30% 1C1 and 30% IC3. Two patients received postoperative chemotherapy. Differentiation between benign MOT and mOC is crucial. A multidisciplinary approach, involving clinician, radiologist and pathologist, helps in clinching the diagnosis. mOC present at a younger age and fertility-sparing procedures are reasonable options in early-stage MOT.

There is limited data on the impact of treatment interruptions due to nonadherence in patients with chronic phase chronic myeloid leukemia (CP-CML) treated with Imatinib. We looked at factors (including adherence to therapy) affecting the outcome in a large cohort of patients with CP-CML. All the 516 patients received Imatinib free-of-cost through a company sponsored scheme, which mandated regular three monthly visits for drug procurement. Data regarding the disease characteristics, adherence to treatment and outcomes, were obtained from patients records. Unwarranted interruption of treatment for more than 1 week was defined as nonadherence. With a median follow-up of 39 months, the estimated 5-year event free survival (EFS) was 70.8% (95%, CI = 63.3-78.3). Nearly one-third of the patients (29.6%) were found to be nonadherent at some point during their treatment. On univariate analysis, the factors adversely affecting the EFS were prolonged symptom duration before diagnosis, treatment with hydroxyurea for more than 1 month before start of Imatinib, and nonadherence to therapy. Only nonadherence was significant in multivariate analysis (HR1.6; P = 0.048). The 5-year EFS in adherent and nonadherent patients was 76.7% and 59.8% respectively (P = 0.011, log rank test). Nonadherent patients were less likely to achieve complete cytogenetic responses (26% versus 44%; P = 0.004; χ(2) test) at any point. A significant proportion of patients with CP-CML have drug interruptions due to nonadherence during therapy and this compromises the EFS. Adherence to therapy must be included as an important evaluation parameter in all future studies of CML.

Aim : This study was designed to retrospectively review our experience with the multimodality management of hepatoblastomas (HB). Materials and Methods: Thirteen patients were treated for HB between 2000 and 2007. The clinical presentations, chemotherapy tolerance and response, surgical procedure undertaken, and complications were analysed. Results: Median age of the population was 12 months (3-60 months), with a male-to-female ratio of 3.3:1. Nine patients were treated with neoadjuvant chemotherapy incorporating cisplatin and adriamycin. Primary surgery was done in four patients. Extent of hepatic resection in the operated patients varied. Mixed type was the predominant histopathological diagnosis. Adjuvant chemotherapy was well tolerated with no morbidity or mortality. Five-year event-free survival (EFS) and overall survival (OS) of all the 13 patients is 76.9%. All the nine patients who could complete multimodality treatment are alive with no evidence of disease or complications with median follow-up of 63 months (46-122 months). Conclusions: Treatment of HB with multidisciplinary approach was well tolerated. OS and EFS of patients were comparable with published studies.

Anemia in tuberculosis is usually anemia of chronic disease. Severe hemolytic anemia is exceedingly rare in tuberculosis patients. We report a patient diagnosed with tubercular lymphadenitis complicated by Coomb's positive hemolytic anemia. Patient responded well to antituberculous treatment. Hematological parameters improved after initiation of antituberculosis treatment. To the best of our knowledge, this is the first case from India of an adult patient with tuberculous lymphadenitis presenting with Coomb's positive hemolytic anemia.

Ganglioneuroma is the benign representative of peripheral neuroblastic tumors, with localized and predominant thoracic presentations in older children. They often have an excellent outcome with surgery alone. A 12-year-old girl presented with an incidentally detected abdominal mass, which was clinicoradiologically a renal mass. Laparotomy revealed a mass adherent to the anterior surface of the right kidney. The mass was carefully dissected out, sacrificing a portion of the inferior vena cava. Histopathological diagnosis was that of a ganglioneuroma. She was kept under follow up.

Background: Primary mediastinal malignancies are uncommon. They can originate from any mediastinal organ or tissue but most commonly arise from thymic, neurogenic, lymphatic, germinal or mesenchymal tissues. Objectives: The aim of this study was to review the clinical presentations, diagnostic methods adopted, the histologies and the treatment outcomes of this rare subset of tumors. Materials and Methods: Case records of 91 patients in the period 1993-2006 at our institute were retrospectively analyzed. Patients with primary mediastinal mass and supraclavicular nodes were included for the analysis. Patients with primary, extrathoracic disease of the lung and peripheral adenopathy were excluded. Actuarial method was used for calculating the disease-free survival and overall survival. Results: Primary mediastinal tumors were seen commonly in males with mean age of 37.48 ± 17.04 years. As many as 97% of patients were symptomatic at presentation. Superior venacaval obstruction (SVCO) was seen in 28% of the patients. As many as 50% of the patients were diagnosed by a fine-needle aspiration or Trucut biopsy, while 28% of the patients required thoracotomy for a diagnosis. Majority of the tumors had anterior mediastinal presentation. Pleural effusion was seen in 20% of the patients, but diagnosis was obtained in only 1%. In adults, thymoma (39%), lymphoma (30%) and germ cell tumor (15%) were the common tumors. In the pediatric population, lymphoma, PNET and neuroblastoma were the common tumors. The 5-year DFS and OS are 50% and 55%, respectively. Conclusion: Primary mediastinal tumors are a challenge to the treating physician because of their unique presentation in the form of medical emergencies, like superior venacaval obstruction and stridor. Diagnosis may require invasive procedures like thoracotomy. Treatment and outcome depend on the histologic subtypes.

To evaluate the efficacy and toxicity of etoposide, methotrexate, actinomycin D, cyclophosphamide and vincristine (EMA-CO) chemotherapy for the treatment of high-risk gestational trophoblastic neoplasia (GTN).Thirty-five patients with high-risk GTN were treated with 196 cycles of EMA-CO between 1997 and 2006. Twenty-nine patients received EMA-CO in the primary setting and another 6 after failure of single-agent chemotherapy. Salvage chemotherapy was offered to selected patients.Of the 29 patients treated with EMA-CO in the primary setting, 22 (75.8%) had a complete clinical response, 5 (17.1%) progressed, and 2 (7.1%) had early deaths. Three patients relapsed after achieving initial complete response. Five were treated with salvage chemotherapy, of which only 2 survived. This translated to overall survival rate of 71% in the primary setting. Five of the 6 patients treated with EMA-CO as second line are survivors. Life threatening toxicity was not seen after EMA-CO. Nine subsequent normal pregnancies were reported after EMA-CO.EMA-CO was highly effective for the management of high-risk GTN, and the toxicities were minimal. Reproductive outcome after treatment with EMA-CO was excellent.

Objective To evaluate the efficacy of etoposide, cisplatin–etoposide, methotrexate, actinomycin-D (EP–EMA) chemotherapy as the frontline treatment for gestational trophoblastic neoplasia (GTN) patients with very high (≥ 12) FIGO prognostic scores. Methods Nine patients with very-high-risk GTN were treated with EP–EMA at the Cancer Institute, Adyar, India, between January 1, 2001, and December 31, 2007. Salvage chemotherapy, adjuvant surgery, and radiotherapy were used when indicated. Clinical response, toxicity, and survival were analyzed separately. Results The median FIGO score was 15. Six (66.7%) patients had a complete clinical response, whereas progressive disease occurred for 3 (33.3%) women. None of the patients relapsed. This translated to an overall survival rate of 66.7% in the primary setting. All patients with liver-only metastases were survivors after treatment with EP–EMA. Grade 3 neutropenia was detected in 3 (33.3%) patients only. No life-threatening toxicity was observed after EP–EMA treatment. Conclusion EP–EMA was highly effective for the primary management of very-high-risk GTN.

Dendritic cell neoplasms are rare malignancies described with an increased frequency. They often involve the lymph nodes, but extranodal presentations are also described. The disease often has an indolent course. Surgery is the main modality though chemotherapy and radiation have also been tried. We present here a case of a 69-year-old male diagnosed to have follicular dendritic cell sarcoma of the neck which had a rapid and fatal course inspite of chemotherapy and radiotherapy. More studies are required to assess the biology of this rare tumor.

Background : The incidence of breast cancer in young patients less than 35 years is less than 1%. The physical and psychosocial morbidity may affect their ability to successfully function in their social roles. Hence we studied the quality of life (QOL) issues in this subset. Materials and Methods :Younger women with age less than 35 years, diagnosed with non-metastatic breast cancer at our Institute, from 1995 to 2005, were included in the study. Quality of life issues were studied during the follow-up using EORTC QOL C30 and BR23. Descriptive and inferential statistics were used in order to analyze the data. Results : A total of 51 patients were included for the study. The mean age at diagnosis was 30 years. The effect of breast cancer on the occupation and marital status was minimal. The global health status and the functional scores were high, while the overall sexual function was lower. The global health status was higher in the mastectomy group. The arm symptoms (P = 0.027) and pain were higher in the Breast conservation surgery (BCS) group. The sexual symptoms appeared to be higher in the ovary ablated group when compared to the ovary preserved group. The sexual functional scores (P = 0.02) and sexual enjoyment scores (P = 0.003) were better in the mastectomy group. Conclusion : The overall QOL in younger patients with breast cancer appeared to be good. The QOL and sexual function were marginally worse in the breast conservation group when compared to mastectomy group.

L-asparaginase has become an integral part in the treatment of acute lymphoblastic leukemia. The major worry of using L-asparaginase is thromboembolism. The case presented here is a 21-year-old lady who developed fatal cortical venous thrombosis during induction phase of treatment for ALL. Early recognition is very important to treat this potentially catastrophic yet treatable complication.

Central nervous system (CNS) being a sanctuary site, intrathecal methotrexate (IT MTX) has become a cornerstone for CNS prophylaxis for acute lymphoblastic leukemia. Recently we noticed eight pediatric patients developing transient neurological event following IT MTX. All of them were acute lymphoblastic leukemia (ALL) patients. Four were high risk patients and others were standard risk (based on total count and age). All of them received BFM 86 protocol. Seven were females. Their age ranged from 4–14 years and the median age was 11.5 years. All the patients had normal CSF studies. Five of them received IT MTX along with intravenous high dose MTX (HDMTX) 5 gm/m before the event. Two received cytosine arabinoside (AraC), (75 mg/m for 4 days) with IT MTX on day one. Only one received cranial irradiation before the event. All the patients developed the event between Day 11 to D14 of IT MTX. Seven patients had hemiparesis and one had quadriparesis. The neurological event recovered within 24 hours in seven of them and within 72 hours on the others. Cerebrospinal fl uid (CSF) study was normal in all patients. All of them were exposed to multiple doses of IT MTX prior to the event. Preservative free MTX recommended dosage (12 mg) was used for all patients and proper administration technique under sterile precautions was ensured. Imaging studies were conducted in all patients within 24 hours of the onset of the event and all had normal standard MR imaging study of the brain. The imaging studies were not repeated later. The incidence of various neurological events following IT-MTX is estimated around 4–15% [1]. Three distinct clinical patterns of neurotoxicity have been observed (a) an acute toxicity, occurring within hours of IT chemotherapy, probably resulting from chemical arachnoiditis; (b) sub acute toxicity occurring within days or weeks, and characterized by symptoms of seizures, transient paresis, or cerebellar abnormalities; and (c) a delayed leukoencephalopathy form that is more commonly observed when IT MTX is used with cranial irradiation [1]. The mechanism of methotrexate induced neurotoxicity is poorly understood. Both high-dose intravenous MTX and intrathecal MTX are proposed to have association with demyelination, white matter necrosis, loss of oligodendroglia, axonal swelling, microcystic encephalomalacia, and atrophy relatively selective for the deep cerebral white matter [2]. An altered myelin metabolism disturbance induced by S. Cyriac · T. G. Sagar · R. Rajendranath Department of Medical Oncology, Cancer Institute, 18 Sardar Patel Road, Guindy, Chennai 600 036, India

The incidence of eosinophilia in Hodgkin lymphoma is approximately 15%. Both peripheral and tissue eosinophilia have been noted in Hodgkin lymphoma. Eosinophils have important role in pathobiology of Hodgkin lymphoma. The mechanism of eosinophilia remains unknown though various mediators like IL-5 and GM-CSF have been implicated. We present a case who was diagnosed to have Hodgkin lymphoma and hypereosinophilia.

Malignant ectomesenchymomas are rare tumors. This tumor affects predominantly young children. Most common site is head and neck. A multi modality approach should be appropriate for this soft-tissue tumor. We present a 43 year old female with malignant ectomesenchymoma of the nasal cavity.

Aims: The aim of the present study is to analyse the outcome and genotypic pattern of metastatic GIST patients which is largely unknown in India. Materials and Methods: The present study was a retrospective analysis of 24 patients of metastatic GIST. The case records were analysed for clinical profile, treatment response and prognostic factors. The archival samples were retrieved for c-kit mutation analysis in all but 5 patients for mutation analysis. Results: The median age of the study population was 56 years. At a median follow up of 29 months, the PFS was 45% at 2 years. Activating c-kit mutations were detected in 10 cases (52.6%). 80% of the mutations were located in Exon 11. Conclusions: The outcome of metastatic GIST patients has definitely improved from a virtually incurable state to a disease where median OS has reached 60 months. The genotype of Indian patients with GIST may be different from the western population which needs to be confirmed in a larger study.

Ataxia telangiectasia (AT) is a rare autosomal recessive disease resulting in progressive degeneration of multiple systems in the body. Both A-T homozygote and heterozygote are at increased risk of developing malignancy. We report a family in which three generations were affected by this disorder. Our index case is a 12-year-old female child, born of second degree consanguineous marriage diagnosed to have ataxia telangiectasia at the age of four years, now presented with fever and neck swelling of one month duration. Family history suggestive of ataxia telangiectasia in maternal uncle and younger sibling was present. History of premature coronary artery disease and death in paternal grandfather was present. On evaluation, child was diagnosed to have Alk negative anaplastic large T cell lymphoma. Management included genetic counseling, examination of all the family members, identification of A-T homozygote and providing appropriate care, regular surveillance of the heterozygote for malignancy.

A 55-year-old woman presented to hospital with a 2-month history of facial puffiness, constipation, hoarse voice, fatigue and cold intolerance. She had no history of illness, and she was not taking any medication. On examination, her vital signs were normal, and she was not in distress. Her voice