Fouzia Siraj

Gastrointestinal mucormycosis—four cases with different risk factors, involving different anatomical sites

Mucormycosis of the gastrointestinal tract is a rare infection that usually occurs in patients who are immunocompromised and carries a high mortality. We report four cases of gastrointestinal mucormycosis seen over a one year period with different presentations, risk factors and different anatomical sites of involvement. A preoperative diagnosis was made only in one patient. All underwent surgery, three survived and one died postoperatively from multiorgan failure.

Successful treatment of multicentric Castleman's disease accompanying myeloma with bortezomib.

Castlemans disease represents an atypical lymphoproliferative disorder which is non-clonal but can turn malignant in the form of lymphoma, Kaposis sarcoma or plasma cell dyscrasia. It has been reported in association with diseases like polyneuropathy organomegaly endocrinopathy monoclonal gammopathy skin changes syndrome and myeloma and very rarely it can be associated with cutaneous vasculitis as in our case. It relatively runs an aggressive course and has a shorter survival. No standard of care therapy is yet established for multicentric Castlemans disease accompanying myeloma. We successfully treated one such patient with bortezomib-based therapy.

Gliosarcoma with prominent smooth muscle component (gliomyosarcoma): A report of 10 cases

BACKGROUND AND AIM Gliosarcoma (GS) is an uncommon malignant tumor of the brain, consisting of malignant glial, usually a glioblastoma (GB), as well as sarcomatous component; the latter is usually in the form of fibrosarcoma. We report a series of 10 GSs with prominent smooth muscle component, which is a rare occurrence. SETTINGS AND DESIGN Out of a series of 225 cases of GB admitted in our hospital, 10 were diagnosed as GS with prominent smooth muscle component, gliomyosarcoma (GMS). MATERIALS AND METHODS This is an observational study based on the experience with 225 cases of GB, encountered between 1995 and 2008, in our hospital. The tumors showing prominent spindle cell component were stained with reticulin and 20 with strongly positive reticulin stain were diagnosed as GS. They were further studied by immunohistochemical staining for glial fibrillary acidic protein (GFAP), smooth muscle actin (SMA), desmin and factor VIII antigen. RESULTS Out of 225 cases of GB, 20 were diagnosed as GS. Ten of these showed prominent smooth muscle component and were diagnosed as GMS. They revealed varying degrees of SMA and factor VIII Ag positivity. In the sarcomatous component, SMA and factor VIII positive cells were seen close to the vessel walls as well as away from them. CONCLUSION GMS containing prominent smooth muscle component may not be as rare as has been reported in the literature. Both GS and GMS appear to arise from the vessel wall at least in some cases, suggesting their possible vascular origin.

Invasive thymoma with osseous metaplasia and cystic change in a case of myasthenia gravis: a rare presentation

A 35-year-old woman, a known case of myasthenia gravis, was found to have an anterior mediastinal mass, which was surgically removed. The preoperative clinical and radiologic diagnosis was that of a thymoma, but foci of calcification and prominent cystic change suggested the remote possibility of a teratoma. Histopathologically, it was confirmed to be a type B3 invasive thymoma with intratumoral ossification. Up to the present, three cases of thymoma with osseous metaplasia, including only one with myasthenia gravis, have been reported in the English literature. The present case report highlights the rare occurrence of osseous metaplasia in thymomas and the diagnostic challenge that it can pose, especially if it is associated with cystic degeneration.

Extraskeletal osteosarcoma of abdominal wall in a child

Compared with osteosarcoma of bone, extraskeletal osteosarcoma is rare and accounts for approximately 1% to 2 % of all soft tissue sarcomas. Osteosarcoma arising in soft tissues is exceedingly rare in children. The tumor most often affects older adults, involves the lower extremity, responds poorly to chemotherapy, and carries a grave prognosis. The authors describe a 4-year-old girl with an extraskeletal osteosarcoma in the lateral abdominal wall.

Novel insights into multitargeted potential of N′-(4-benzylpiperidin-1-yl)alkylamine derivatives in the management of Alzheimer's disease associated pathogenesis

In response to the molecular complexity of AD, the strategy of multi-target directed ligand (MTDL) holds great potential in modulating different targets involved in the neurodegenerative cascade of AD. In this context, there is a need for the use of privileged scaffolds for discovering potential multifunctional agents for AD treatment. Our group have previously reported a novel series of benzyl piperazine/piperidine based multifunctional agents endowed with in vitro profile appropriate for identifying novel lead candidates with disease-modifying potential. Herein, we have further made an attempt to decipher some of the key mechanisms behind the multitargeted potential of most active inhibitors (5h and 5k) and their characterization for anti-Alzheimer effects. In the present study, investigation of compounds 5h and 5k through CD experiments confirmed their ability in preventing β-sheet aggregation and fibril formation. Morphological visualization in TEM suggested that test inhibitors may interfere with rate of peptide nucleation, leading to short fibrillar aggregates. ThT based fluorimetric assay further demonstrated that 5h and 5k inhibited AChE-mediated Aβ fibrillogenesis via their interaction with the AChE peripheral anionic site. Molecular docking and simulations studies indicated that these compounds could potentially bind to Aβ by inhibiting the toxic conformation of Aβ1–42 and stabilizing the α-helical content. Furthermore, compounds exerted neuroprotective action on SH-SY5Y cells towards Aβ and H2O2-mediated cell death and oxidative injury by inhibiting ROS generation. Moreover, administration of 5h and 5k (5 mg kg−1), significantly reversed scopolamine-induced neurobehavioral, biochemical, neurochemical and histopathological changes in a manner comparable to standard drug donepezil. Together, the present findings provide novel insights into disease-modifying potential of benzylpiperidine derivatives towards the management of AD.

Hepatic Mucormycosis in a Patient of Acute Lymphoblastic Leukemia: A Case Report with Literature Review

Mucormycosis is an invasive opportunistic fungal infection associated with a high mortality rate, and normally occurs in immunocompromised patients. It can be encountered during neutropenia following chemotherapy in acute leukemia patients. The common sites involved are rhinocerebral, pulmonary, gastrointestinal and spleen. Hepatic involvement has been reported rarely. We hereby report a case of acute lymphoblastic leukemia who while on chemotherapy developed hepatic mucormycosis and was managed successfully despite its treatment being quite challenging, especially in the context of surgery in a neutropenic sick patient.

Hidradenoma papilliferum presenting as an eyelid mass.

Hidradenoma papilliferum is a benign adnexal tumor originating from apocrine glands of skin. It is commonly seen in the anogenital region of middle-aged women and presents as an asymptomatic, slow-growing, solitary nodule. Rarely this tumor occurs at extragenital sites and is called ectopic hidradenoma papilliferum. Although head and neck is a common site for this group, only 3 cases in the upper eyelid have been described. We present the first case of ectopic hidradenoma papilleferum presenting as a cystic swelling in the lower eyelid. A brief review of literature is included.

Myeloid sarcoma: a report of four cases at unusual sites

Background: Myeloid sarcoma (MS) or granulocytic sarcoma is a rare tumor consisting of myeloid blasts with or without maturation occurring at anatomic sites other than the bone marrow. MS can involve any organ system but shows a predilection for skin, bone, and soft tissues of head and neck region. Case report: We report four cases of MS occurring at unusual sites, out of which three were de novo and one was associated with acute myeloid leukemia (AML). Conclusion: Although MS is associated with AML, it can rarely present without any existent hematologic disease. Differential diagnosis of a soft tissue mass should include MS even in the absence of leukemia. Establishment of the correct diagnosis depends on morphologic, histochemical, and immunohistochemical examination.

Immunohistochemical expression of chemokine receptor CXCR3 and its ligand CXCL10 in low-grade astrocytomas and glioblastoma multiforme: A tissue microarray-based comparison

UNLABELLED Glioblastoma multiforme (GBM) and diffuse astrocytoma (DA) are the most frequently encountered gliomas. Due to poor prognosis and limited success of the currently available treatment modalities there is a need to identify new therapeutic targets. Chemokines (CKs) regulate cellular functions like chemotaxis, angiogenesis, apoptosis, and cell cycle progression that play role in tumor growth. OBJECTIVE To study comparative immunoexpression of CXCR3 and CXCL10 in DA and GBM using a high-throughput tissue microarray (TMA). MATERIALS AND METHODS A TMA of 1.0 mm core diameter was made from formalin-fixed, paraffin-embedded donor blocks of 25 pilocytic astrocytomas (PA), 45 DA, and 75 GBM. Immunohistochemical staining for CXCR3 and CXCL10 was performed. RESULTS Out of 145, 129 cores were suitable for immunohistochemical evaluation after processing and immunohistochemistry. Strong CXCR3 immunoexpression was observed in 72.7% cases of GBM as compared to 31.8% cases of DA. 50.7% of GBM and 24.5% of DA showed strong immunoexpression of CXCL10. Overall comparisons between DA and GBM for CXCR3 and CXCL10 showed statistically significant correlation between the two with P < 0.001 and P = 0.02, respectively. A positive correlation was observed between CXCR3 and MIB-1. Pearsons correlation coefficient r = 0.548 and 0.330 for DA and GBM, respectively with P < 0.01. CONCLUSION GBM shows overexpression of CXCR3 and CXCL10 in comparison to DA, indicating that they play an important role in tumor growth and progression. Inhibition of this receptor-ligand axis may be a potential therapeutic target for arresting tumor growth and development of a glioblastoma.