Search

Treatment of multi-resistant ovarian cancer is palliative. Patient after receiving multiple lines of chemotherapy are neither in good PS nor have good compliance to receive further toxic treatment. Bevacizumab is a vascular endothelial growth factor which inhibit angiogenesis. Bevacizumab is generally used in combination with chemotherapy. Due to its low toxicity profile, improvement in progression free survival (PFS), easy availability in resources limited settings, it is a good adjunct to more toxic and costly regimens. Development of hypertension is one of the most common adverse event of bevacizumab. We present a case of patient of metastatic carcinoma ovary, who developed grade 4 hypertension post 1st cycle of bevacizumab for which treatment was withheld. After control of blood pressure, she was rechallenged with bevacizumab and patient did not had any hypertensive episode and is doing well at present.

Hepatocellular carcinoma (HCC) is the most common, malignant tumor of the liver. Hepatocellular carcinoma (HCC) commonly metastasizes to the lungs, abdominal lymph nodes, adrenal glands, or bones. Distant lymph node metastases are rare in hepatocellular carcinoma. A 49-year-old male patient presented with right sided neck mass. On examination there was right cervical lymphadenopathy and hepatomegaly. Excisional cervical lymph node biopsy showed metastatic carcinoma. However, further examination of the biopsy specimen for immuno-histochemistry markers, shows positivity for HepPar-1 & CD-10 suggestive of hepatocellular carcinoma. Considering the high incidence of HCC in Asia, a special attention should be given to such unusual site of presentation and metastasis of HCC; therefore, not to miss the diagnosis.

Esophageal lymphoma is a rare condition, accounting for less than 1% of all gastrointestinal lymphomas. Primary extra nodal esophageal lymphoma constitutes less than 0.2% cases of the total esophageal lymphomas. The definition of primary GI lymphoma has differed among authors. The etiology of the disease is unknown, with the role of Epstein-Barr virus being controversial. The common symptoms of patients with esophageal lymphoma include dysphasia, odynophagia, weight loss, chest pain or present as a result of complications. Burkitt’s lymphoma is one of the fastest growing human malignancies, with a 100% replication rate. Endemic, sporadic (non-endemic) and immunodeficient variants have been recognized. The diagnosis of Burkitt’s lymphoma relies on morphologic findings, immunophenotyping results, and cytogenetic features. Burkitt’s lymphoma is usually treated with LMB-96 protocol depending on the risk stratification. We present a case of primary esophageal Burkitt’s lymphoma, which has been successfully treated with LMB-96 protocol. An extensive review of literature did not reveal a single case of esophageal Burkitt’s lymphoma. To the best of our knowledge this is the first case report in the world literature with diagnosis of primary esophageal Burkitt’s lymphoma.

Treatment related acute myeloid leukemia (t-AML) is well documented phenomenon after chemotherapy. In this subgroup of patients acute promyelocytic leukemia (APML) due to delayed complication of using anthracycline is very rare occurrence. Very few cases are reported in world literature. We are reporting a rare case of occurrence of t-APML in cured breast cancer patient treated with doxorubicin. 43 year old female presented with triple negative early breast cancer treated initially with Right modified radical mastectomy. Pathological staging was pT2N0M0. She was treated with 6 cycle of adjuvant AC (Doxorubicin, Cyclophosphamide). After latent period of 23 months she developed symptoms of fever, weakness and generalized body ache. On further investigation she was found to have acute promyelocytic leukemia (APML). We had successfully treated t-APML with conventional 7+3 induction and subsequent consolidation with ATRA (All Trans Retinoic Acid) and arsenic trioxide. Patient was given maintenance treatment for 18 months after confirming negative PML RARA by RT PCR and declared cured. Patient is under regular surveillance in our centre.

Myeloid sarcoma (chloroma, granulocytic sarcoma, or extramedullary myeloid tumour) is an extramedullary mass forming neoplasm composed of myeloid precursor cells. It is usually associated with myeloproliferative disorders but very rarely may precede the onset of leukemia. Here, we are presenting a rare case of primary vaginal myeloid sarcoma in a geriatric female patient without initial presentation of acute myeloid leukemia (AML). A 68-year-old female patient with ECOG Performance Score of 1 presented with pervaginal bleeding for 20 days. On colposcopic examination, she was found to have mass in the anterior fornix of vagina. A punch biopsy specimen revealed chloromatous infiltration of the vagina. LCA (leukocyte common antigen), MPO (myeloperoxidase), and c-kit were strongly positive on IHC (immunohistochemistry). The patient’s routine blood investigations were normal including peripheral smear, lactose dehydrogenase, uric acid, 2D echocardiography, conventional cytogenetics, bone marrow aspiration, and biopsy. The patient was given 4 cycles of decitabine (Decitex, manufactured by Sun Pharmaceutical Industries Limited, India), 20 mg/m2 for 5 days at an interval of 28 days. There was a partial response to decitabine according to RECIST criteria. As decitabine therapy was well tolerated, we are continuing in the same way until disease progression without any complications. The patient is undergoing regular follow-up at our centre.

The most frequent form of congenital dyserythropoiesis (CDA) is congenital dyserythropoietic anemia II (CDA II). CDA II is a rare genetic anemia in humans, inherited in an autosomally recessive mode, characterized by hepatosplenomegaly normocytic anemia and hemolytic jaundice. Patients are usually transfusion-independent except in severe type. We are here reporting a case of severe transfusion-dependent type II congenital dyserythropoietic anemia in a 5-year-old patient who has undergone allogeneic hematopoietic stem cell transplantation (HSCT) at our bone marrow transplantation centre. Patient has had up until now more than 14 mL/kg/month of packed cell volume (PCV), which he required every 15 to 20 days to maintain his hemoglobin of 10 gm/dL and hematocrit of 30%. His pre-HSCT serum ferritin was 1500 ng/mL and he was on iron chelating therapy. Donor was HLA identical sibling (younger brother). The preparative regimen used was busulfan, cyclophosphamide, and antithymocyte globulin (Thymoglobulin). Cyclosporine and short-term methotrexate were used for graft versus host disease (GVHD) prophylaxis. Engraftment of donor cells was quick and the posttransplant course was uneventful. The patient is presently alive and doing well and he has been transfusion-independent for the past 33 months after HSCT.

Primary mediastinal sarcomas are aggressive tumors with a very rare incidence. This report describes the case of a 35-year-old male patient who presented with acute symptoms of dyspnoea, facial puffiness, voice-hoarseness, and engorged neck veins. With the clinical picture consistent with the superior vena cava (SVC) syndrome, the patient was investigated with computed tomography of the chest. This revealed a large soft tissue density mass lesion compressing the SVC along with other critical superior mediastinal structures. Histopathological evaluation of the mass revealed features consistent with a soft tissue sarcoma and positive staining was observed for vimentin and S-100. Cytogenetic analysis by fluorescent in situ hybridisation (FISH) demonstrated the t(X:18) translocation. Thus diagnosis was established as primary mediastinal synovial sarcoma. Patient was treated with three cycles of neoadjuvant chemotherapy, to which there was a partial response as per the RECIST criteria. Surgical excision of the mediastinal mass was performed, and further postoperative treatment with adjuvant chemoradiotherapy was provided. Patient currently is free of disease. This is to the best of our knowledge the first report in the world literature of a successfully treated case of “primary mediastinal sarcomas presenting as SVC syndrome.” Patient is under regular surveillance at our clinic and remains free of recurrence one year after treatment completion.

Synovial sarcomas (SS) are classified as subgroup of soft tissue sarcomas affecting mainly extremities of young adults. Primary SS of kidney are very rare tumours with poor prognosis. Though they have characteristic histology and immunohistochemistry (IHC) due to rarity of incidence it is difficult to diagnose them. Sometimes chromosomal rearrangement studies are required to confirm the diagnosis. We are presenting a case of 41-year-old male who was referred to our cancer centre for evaluation of left renal mass. CT scan of abdomen revealed a large left renal mass encasing the aorta. Biopsy of renal mass revealed poorly differentiated sarcoma and IHC was positive for vimentin, CD99, and BCL2 and negative for AE1, epithelial membrane antigen, and leukocyte common antigen. The patient was clinically inoperable as renal mass was encasing the aorta. So he was subsequently offered palliative chemotherapy in form of ifosfamide and adriamycin. CT abdomen shows partial response after 3 cycles of chemotherapy according to RECIST criteria.

Hepatoblastoma (HB) is a rare malignant tumour of the liver and usually occurs in the first three years of life. Hepatoblastoma in adolescents and young adults is extremely rare; nevertheless the prognosis is much worse than in childhood, because these kinds of tumours are usually diagnosed late. Characteristic imaging and histopathological and AFP levels help in the diagnosis of hepatoblastoma. Paraneoplastic features of hepatoblastoma are not uncommon at presentation and include erythrocytosis, thrombocytosis, hypocalcaemia, isosexual precocious puberty, and rarely hypoglycaemia. Even though hypoglycaemia is commonly seen in hepatocellular carcinoma, its association with hepatoblastoma is very rare. We present a case of 15-year-old male patient presenting with complaints of recurrent hypoglycaemic seizures ultimately leading to diagnosis of hepatoblastoma. Managed successfully with neoadjuvant chemotherapy, surgery and adjuvant chemotherapy with adriamycin and cisplatin based regimens. An extensive review of literature in the PubMed and MEDLINE did not reveal much data on paraneoplastic recurrent hypoglycaemic seizures as an initial presentation of hepatoblastomas in adolescents and young adults.