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An unusual case of extramedullary relapse in a known case of T cell lymphoblastic lymphoma is presented here. The patient is a 24-year-old girl diagnosed with T cell lymphoblastic lymphoma in June 2020. The patient th showed extramedullary relapse in the gastrointestinal mucosa without bone marrow recurrence whilst on the 6 month of BFM (Berlin Frankfurt Munster) -90 maintenance. Isolated gastrointestinal infiltrate is unusual at presentation or relapse of T cell lymphoblastic lymphoma. While on BFM-90 maintenance, she presented with multiple vomiting and abdominal pain episodes. Upper gastrointestinal scopy revealed multiple gastric ulcers, with morphology and immune-phenotyping identical to her initial T cell lymphoblastic lymphoma. We could not find evidence of leukaemic activity in the blood, cerebrospinal fluid or bone marrow. Several types of leukemic infiltrates have been recognised at post-mortem examination; the fact that makes our case is unique is T cell lymphoblastic lymphoma presenting as an isolated malignant ulcer, which to the best of our knowledge, has not been reported. We conclude that relapsed T cell lymphoblastic lymphoma may present with gastrointestinal infiltration. Further investigations are warranted to establish the same
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Background: The rationale of this study is to reveal the statistics of pediatric chronic myeloid leukemia (CML) patients. Subjects and Methods: It is a retrospective analysis conducted to assess pediatric CML data from January 1998 to December 2014. There are 65 (3.2%) pediatric CML patients out of entire 2008 patients of CML. Data were analyzed regarding epidemiological characteristics, clinical presentations, response and side effects of imatinib, event-free survival, and overall survival of the pediatric CML patients. Results: The median age of diagnosis was 11.84 years, and 76.9% patients were male and 23.07% patients were female. Sixty (92.3%) patients were in CML-chronic phase, 3 (4.6%) patients in CML-accelerated phase, and 2 (3.07%) patients in CML-blastic crisis. Most common initial symptoms and signs are weakness (60.0%), abdominal pain (55.38%), splenomegaly (100%), and hepatomegaly (86.5%). 67.3% of patients have white blood counts <100 × 109/L and 92.3% had platelets >150 × 109/L. In the initial months of 2002, imatinib was available and utilized in 54 patients. Of 54 patients, complete hematological response at 3 months, partial cytogenetic response at 6 months, complete cytogenetic response at 12 months, and major molecular response (MMR) at 18 months were 77.77%, 59.2%, 48.14%, and 40.74%, respectively. MMR at 36 months was 62.96% ( n = 34). Most common imatinib-related side effects are gastrointestinal upset and myelosuppression. Conclusion: Pediatric CML in India is comparable with Western countries regarding epidemiological characteristic, clinical presentations, and tolerance of imatinib. As there is a paucity of universal literature regarding pediatric CML (especially data from Southeast Asian region), this article may fill up that space
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Background: Gestational trophoblastic neoplasia (GTN) was earlier a dreaded malignancy with high mortality rates. GTN is now considered to be one of the most curable solid tumours in women with cure rates greater than 90% even in the presence of metastases. Despite the high chemo sensitivity, treatment failure or drug resistance has been described in both groups.Methods: In this study, available records of GTN cases over 6 years were reviewed with emphasis on those who were resistant to the first line of chemotherapy. Of these, 37(34.58%) were resistant to the first line of chemotherapy. These cases were studied with respect to age, parity, antecedent pregnancy, interval from antecedent pregnancy, pretreatment β hCG, risk score and presence of metastases. The data was analyzed in order to find any risk factors associated with chemo-resistance.Results: Total number of cases of GTN was 107. Out of these 107 cases, 63 (58.88%) were low risk and 44 (41.12%) were high risk according to FIGO scoring system. Complete response was achieved with first line chemotherapy in 70 (65.42%) patients. The remaining 37 (34.57%) were resistant to first line chemotherapy. In the low risk group, 30 (47.62%) cases, and in the high-risk group, 7(15.91%) were resistant to first line of chemotherapy.Conclusions: Despite the high chemo sensitivity of GTN, resistance to first line chemotherapy may be encountered in up to 40% of cases. It is important to identify the patients who are at risk to develop resistance, early identification of resistance and change of chemotherapy so as to minimize the exposure of these patients to ineffective chemotherapy.
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Background & objectives: The efficacy and toxicity of a given chemotherapy regimen varies widely among patients due to the inherited variability of genes that are involved in drug metabolism. There are several crucial enzymes identified involving metabolism of 5-fluorouracil (5-FU) and cisplatin, which are polymorphic. We studied head and neck cancer patients (n=23) on 5-FU and cisplatin combination therapy attending a tertiary care cancer research institute in Gujarat, India, to understand the effect of a particular genotype on toxicity. Methods: The patients were genotyped for dihydropyrimidine (DPYD) (85T>C, IVS14+1G>A, 2846A>T, 2194G>A), thymidylate synthase (TYMS) [28bp tandem repeat in the promoter enhancer region (TSER)], methylenetetrahydrofolate reductase (MTHFR) (677C>T, 1298A>C), glutathione S-transferase P1(GSTP1) (Ile105Val), glutathione S-transferase T1 (GSTT1) (null allele) and glutathione S-transferase M1 (GSTM1) (null allele) by multiplex allele-specific PCR and long range PCR. Results: Of the 23 (19 males 4 females, age range 18-16 yr) patients, two had grade 3 and 4 toxicity while the remaining 21 had 0 to 2 grade toxicity after treatment with 5-FU and cisplatin combination therapy. An association between the genotype of GSTM1 (+/- and -/-) and the toxicity of cisplatin (P=0.043) was observed. Interpretation & conclusions: The findings of this preliminary study suggested an association between the variants of GSTM1 and toxicity observed due to cisplatin. Well planned studies on a large sample of head and neck cancer patients need to be conducted to understand the effects of these genetic variants on toxicity and efficacy of anticancer drugs.
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A case of primary chondrosarcoma of the left lung in 50 year-old man is presented. The tumor was diagnosed as primary chondrosarcoma of the lung after exclusion of any primary lesion elsewhere. Histologically, tumor consisted of predominantly chondromatous lesion. Immunohistochemistry showed that tumor cells positive for S-100 protein and vimentin, and negative for epithelial markers. On the basis of clinical, histological and immunohistochemical studies, the tumor was diagnosed as a primary chondrosarcoma of the lung.