Inflammatory Myofibroblastic Tumor With Rapid Recurrence and Distant Metastasis: Report of a Rare Case.

Inflammatory myofibroblastic tumors (IMTs) are rare spindle cell tumors clinically, morphologically, and genetically heterogeneous, mimicking many other reactive and neoplastic lesions and creating great diagnostic problems. Although it is generally characterized by oncogene-derived proliferation of myofibroblasts in a background of polyclonal inflammatory cell infiltrates, morphological variations do occur requiring immunohistochemistry and molecular genetics to confirm the diagnosis. It encompasses a wide age range, and locations, mostly said to be of intermediate grade having a low risk of recurrence and metastasis. However, its biological behavior and course are variable and unpredictable. Here, we report a case of thoracic IMT in a 32-year-old adult female presenting with a history of fever, cough, and chest pain associated with neutrophilic leukocytosis. Radiological investigations revealed a large mass in the thoracic region with possibilities of hydatid cyst and neurogenic tumor. Initial core needle biopsy specimen and subsequent local resection specimen revealed the diagnosis of IMT on histopathology and immunohistochemistry, having conventional morphology with expression of Anaplastic lymphoma kinase (ALK) protein. The patient developed rapid local recurrence and was started with first-generation ALK inhibitor Crizotinib. After a brief period of response, she developed vertebral and brain metastasis within a short span of time and was switched to a third-generation ALK inhibitor, Lorlatinib. The patient is on regular follow-up, has stable disease, and maintains a good quality of life after two years of diagnosis.

Response assessment of patients with chronic myeloid leukemia receiving imatinib mesylate (Glivec) therapy: experience from a single center in a developing country.

Imatinib mesylate, a targeted inhibitor of BCR-ABL tyrosine kinase, is a standard of care for chronic myeloid leukemia (CML). There are few publications on responses of patients with CML from the Indian subcontinent. This study analyzed the response rate, progression-free survival (PFS), overall survival (OS), and toxicities in patients with CML given imatinib. Analysis included patients with CML who received imatinib under the GIPAP program at our institution from January 2002 to December 2008. Standard criteria for hematological and cytogenetic responses were used. There were 400 patients, with a median follow-up of 47 months. One hundred and seventy received prior non-imatinib therapy and 230 patients received imatinib upfront. Ninety-five percent of patients achieved complete hematological response. The cumulative best rate of major cytogenetic response was 72%, with 53% complete cytogenetic response and 19% partial cytogenetic response. The estimated PFS and OS at median follow-up for the whole group was 76% and 94%, respectively. Differences in PFS and OS in prior non-imatinib and upfront imatinib groups were not statistically significant. However, better PFS and OS were seen in the upfront imatinib group. Imatinib was well tolerated in our study.

Plasma cell leukemia--a study of 28 cases from India.

Plasma cell leukemia (PCL) is a rare neoplasm that has not been comprehensively reported in an Indian population. We report the clinico-pathological features of 28 cases studied during 1999-2008. Organomegaly and bleeding tendency was common in primary PCL but not in secondary. Misdiagnosis as acute leukemia or the leukemic phase of lymphoma on the initial peripheral blood smear examination was frequent (31.4% cases) in the primary form of PCL. This is best addressed by an emphasis on the morphological appearances and confirmation by simple serum electrophoresis rather than by more sophisticated testing that may not be widely available. Response to treatment is poor and PCL has a poor prognosis, a situation that may be amenable to improvement by a better understanding of the biology of the disease.

Subcutaneous panniculitislike T-cell lymphoma with hemophagocytosis: complete remission with BFM-90 protocol.

SUMMARY: Subcutaneous panniculitislike T-cell lymphoma (SPTCL) is an uncommon type of cutaneous lymphoma. In many cases, SPTCL is accompanied by hemophagocytic syndrome (HPS), resulting in prominent systemic symptoms. The natural history, optimal treatment strategy, and prognostic factors associated with this malignancy are not well defined. We report an 11-year-old boy of SPTCL with HPS who was initially treated with conventional cyclophosphamide, doxorubicin, vincristine, and prednisone chemotherapy, but progressed later on therapy. Subsequently, the child was treated with multiagent combination chemotherapy as per BFM-90 protocol and achieved complete remission, and has remained so for 3 years. This report suggests the value of this particular multiagent combination chemotherapy regimen in the treatment of patients with SPTCL and HPS.

Proton magnetic resonance spectroscopy of tubercular breast abscess: report of a case.

In vivo proton magnetic resonance spectroscopy (H-MRS) is a functional imaging modality. When magnetic resonance imaging is coupled with H-MRS, it results in accurate metabolic characterization of various lesions. Proton magnetic resonance spectroscopy has an established role in evaluating malignant breast lesions, and the increasing number of published literature supports the role of H-MRS in patients with breast cancer. However, H-MRS can be of help in evaluating benign breast disease. We present a case of tubercular breast abscess, initial diagnosis of which was suggested based on characteristic lipid pick on H-MRS and was subsequently confirmed by fine needle aspiration biopsy of the breast lesion.