Isolated Extramedullary Relapse in the Gastrointestinal Mucosa of a Patient with T cell Lymphoblastic Lymphoma

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

Pediatric chronic myeloid leukemia: A single-center experience

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

Isolated acrometastasis: A rare presenting feature of endometrial carcinoma

The most common presenting feature of endometrial carcinoma (EC) is abnormal uterine bleeding. Bone metastasis, as a presenting feature of EC, is very unusual which is usually restricted to pelvis and vertebrae. The occurrence of foot metastasis is exceedingly rare. We report a case of a postmenopausal female presented with pain and swelling involving right foot. Biopsy revealed metastatic adenocarcinoma. The patient denied any history of vaginal bleeding or other gynecological symptoms. Bone scan suggested increased uptake in multiple tarsal bones. Uterine curettage confirmed the diagnosis of endometrial adenocarcinoma. The patient was successfully treated with debulking surgery, palliative radiotherapy to the right foot, bisphosphonates, and systemic chemotherapy with marked improvement in local symptoms and is under follow-up for the last 6 months after completion of the treatment. An extensive review of the literature, to the best of our knowledge, did not reveal many cases of acrometastasis as a presenting feature of EC

Hemangiopericytoma of supraglottis: A rare case report and review of literature

In 1942, Stout and Murray first used the term hemangiopericytoma to describe a tumor which is distinguished histologically from other types of vascular neoplasm characterized by proliferation of pericytes. It is a rare neoplasm that was originally described as a vascular tumor derived from the pericytes. They account for 2%–3% of all soft tissue sarcomas in humans and they occur mainly in the musculoskeletal system. About 15%-30% of all hemangiopericytomas occur in the head and neck region. Hemangiopericytoma of supraglottis is very rare neoplasm with only nine cases reported in literature and ours is the tenth case overall and first case in pediatric age group. Herein, we are presenting an extremely rare case report of hemangiopericytoma of supraglottis in a 6-year-old male child who presented with stridor followed by which tracheostomy was done. Later, the patient was treated initially with radiotherapy followed by surgery, i.e., laryngectomy in view of residual disease postcurative radiotherapy. Hence, hemangiopericytoma is a very rare tumor overall and can present in pediatric age group and can be one most important differential diagnosis because many patients in this age group, stridor most commonly occurs due to the infectious causes such as influenza virus and diphtheria-induced croup, i.e., laryngotracheobronchitis

Clinical trial : A Review.

Randomized clinical trials are scientific investigations that examine and evaluate the safety and efficacy of new drugs, devices, tests, or lifestyle interventions using human subjects. The results that these clinical trials generate are considered to be the most robust data in the era of evidence-based medicine. The primary aim of most clinical trials is to provide an unbiased evaluation of the merits of using one or more treatment options for a given disease or condition of interest. Ideally, clinical trials should be performed in a way that isolates the effect of treatment on the study outcome and provides results that are free from study bias.