Metastatic Small Cell Neuroendocrine Carcinoma of Endometrium: Rare Entity, Rare Presentation.

Primary small cell neuroendocrine carcinoma of endometrium is very rare and aggressive carcinoma. Most patients present with metastases at the time of diagnosis and have very poor prognosis. Only very few cases are reported in literature.

A Commoner at an Extremely Rare Site: A Case of Dorsal Intramedullary Spinal Dermoid Cyst.

Intramedullary dorsal dermoid cysts are rare benign tumors, arising from the nests of embryonic ectoderm, which get buried or trapped under the lines of fusion of the ectodermal folds in the developing embryo. We present a rare case of intramedullary dermoid cyst in a 30-year-old asymptomatic female, who presented with paraplegia and ataxia. Magnetic resonance imaging was suggestive of neoplastic intramedullary mass.

Intracranial Myopericytoma: A Rare Benign Tumor at an Extremely Rare Location.

A 50-year-old female presented with a history of seizures, headache, nausea, and vomiting. On imaging, parafalcine meningioma with mass effect features was rendered. She underwent right frontal tumor excision and craniotomy.

Intracranial Myopericytoma: A Rare Benign Tumor at an Extremely Rare Location.

A 50-year-old female with a history of seizures, headache, nausea, and vomiting. On imaging, parafalcine meningioma with mass effect features was rendered. She underwent right frontal tumor excision and craniotomy.

Adrenocortical oncocytoma associated with androgen excess: A rare cause of hirsutism.

Adrenal oncocytomas are rare neoplasms that are usually benign and nonfunctional, and often detected incidentally. Very few cases have been reported of functioning adrenal oncocytomas. We report a rare case of adrenocortical oncocytoma in a 29-year-old female presenting with hirsutism and irregular menstrual history.

Diffuse midline glioma-H3K27M mutant. A novel entity with a defining and specific IHC marker.

Diffuse Midline Glioma-H3K27M mutant is a specific entity added to the 2016 updated WHO classification of CNS tumours that represents the majority of diffuse intrinsic pontine gliomas, although identical tumours are also found elsewhere in the midline. They are aggressive tumours with a poor prognosis and considered WHO GRADE IV regardless of histological features. Patients with H3K27M-mutant gliomas in unusual anatomical locations have a better prognosis than those with corresponding tumors in the brainstem and this helps in the treatment stratification of diffuse gliomas.

Bilateral alveolar rhabdomyosarcoma of breast: a rare entity.

Sarcomas of breast constitute less than 1% of all malignant breast tumours. Alveolar rhabdomyosarcoma (RMS) is very rare in breast with limited case reports in literature, and primary alveolar RMS arising from breast is still less common than metastatic RMS. Here, we report a case of primary bilateral alveolar RMS of breast in an adolescent female where the correct diagnosis was obfuscated by an overlap in the histological features of RMS and high-grade invasive ductal carcinoma.

CTLA4Ig-primed donor lymphocyte infusions following haploidentical transplantation improve outcome with a distinct pattern of early immune reconstitution as compared to conventional donor lymphocyte infusions in advanced hematological malignancies.

CTLA4Ig has a unique property to spare or even potentiate natural killer (NK) cell-mediated cytotoxicity, whilst inhibiting T cell activation. We explored the efficacy of prophylactic DLI following CTLA4Ig (CTLA4Ig-DLI group, n = 75), compared to conventional DLI (DLI group, n = 50), in patients with advanced hematological malignancies receiving PTCy-based haploidentical transplantation. Acute and chronic GVHD in the CTLA4Ig-DLI group were 9.

CTLA4Ig in an Extended Schedule along with Sirolimus Improves Outcome with a Distinct Pattern of Immune Reconstitution Following Post-Transplantation Cyclophosphamide-Based Haploidentical Transplantation for Hemoglobinopathies.

The major hindrances to the success of a haploidentical hematopoietic cell transplantation for hemoglobinopathies are graft failure, early post-transplant hemophagocytic syndrome (PTHPS), and graft-versus-host disease (GVHD). Following the successful incorporation of CTLA4Ig (abatacept) in post-transplantation cyclophosphamide-based haploidentical transplantation, we piloted this approach in 10 patients (aged 3 to 19 years), with thalassemia major (TM, n=5) and sickle cell disease (n = 5). Pretransplant immunosuppressive therapy (pTIST) was administered for 10 weeks.

Impact of extended infusional mesna prophylaxis on the incidence of BK viruria and hemorrhagic cystitis following post-transplantation cyclophosphamide and CTLA4Ig-based haploidentical transplantation.

Hemorrhagic cystitis (HC) has been reported with increased frequency following post-transplantation cyclophosphamide (PTCy)-based haploidentical hematopoietic cell transplantation (HCT) along with a strong association with BK viruria. We prospectively evaluated the incidence of BK viruria and HC in 115 patients (median age 20 years, 2-65) undergoing PTCy-based haploidentical HCT with (n = 71) or without (n = 44) CTLA4Ig. HC prophylaxis consisted of a continuous infusion of mesna 30 min prior and 48 h post-PTCy.

Metastatic hepatocellular carcinoma to the parotid gland: A diagnostic dilemma with review of the literature.

Hepatocellular carcinoma (HCC) is the most common primary hepatic malignancy and is a leading cause of cancer-related death worldwide. It has a very aggressive clinical course, with a mean survival rate of much less than a year if left untreated. Here, we present a case of a 68-year-old male with progressively enlarging painful right facial swelling, involving the ramus and condyle of mandible on contrast-enhanced computed tomography mimicking an osteosarcoma.

CTLA4Ig-based reduced intensity conditioning and donor lymphocyte infusions for haploidentical transplantation in refractory aggressive B-cell lymphoma relapsing after an autograft: Early results from a pilot study.

CTLA4Ig-primed donor lymphocyte infusions (DLIs) have been found to promote natural killer (NK) cell-mediated anti-leukemia effect following haploidentical hematopoietic cell transplantation (HCT). Incorporation of CTLA4Ig in conditioning aided long-term remission in myeloma probably by blocking the CD28-CD86 pro-survival pathway when combined with CTLA4Ig-primed DLI. We explored a similar approach in 12 patients (8-65 years) who had refractory aggressive B-cell lymphoma (R-ABCL) following autologous HCT.

Cutaneous melanoma in childhood presenting as intraparotid lymph node metastasis.

Cutaneous melanoma in childhood is a rare disease. Rendering a clinical diagnosis of melanoma in pediatric patients is confounded by the fact that pigmented lesions in pediatric patients do not conform to the ABCDE rules applicable to adult patients. Furthermore, making a histologic diagnosis of cutaneous melanoma in childhood is also difficult with no universally accepted criteria applicable to pediatric melanomas.

Melanotic neuroectodermal tumor of infancy: A rare case report.

Melanotic neuroectodermal tumor of infancy is a rare benign but locally aggressive neoplasm of neural crest origin with a high recurrence rate. It usually affects infants of < 1 year of age. Involvement of maxilla of an infant is the most common presentation although cases outside this setting have been reported.

Giant cell tumour of talus with osteosarcomatous transformation in a young male: a case report.

A case of osteosarcomatous transformation in a benign giant cell tumour is described. Spontaneous malignant transformation is rare in a giant cell tumour with only 17 cases reported in the literature till date. In addition, a giant cell tumour arising in the bones of the hands and feet is in itself a rarity.