Devajit Nath

Intraosseous primary malignant peripheral nerve sheath tumor of the calcaneus: an unusual case and review of literature.

Malignant peripheral nerve sheath tumors (MPNSTs) are uncommon sarcomas that originate from a peripheral nerve or neurofibroma either spontaneously or in association with neurofibromatosis type 1. MPNSTs account for approximately 5% of all soft tissue malignancies. The tumor is commonly seen in the extremities and trunk. Most of these tumors are high-grade with the potential to recur and metastasize. Common metastatic sites include the lungs, bone, and pleura. Primary intraosseous MPNST is rare, and the diagnosis of intraosseous MPNST, especially in an unusual location is difficult because of its cellular origin, histomorphological similarities with other sarcomas, and bone is the most common site for metastasis. We report an unusual case of MPNST of the calcaneus in a young male.

Extrapulmonary Small Cell Carcinoma - a Case Series of Oropharyngeal and Esophageal Primary Sites Treated with Chemo-Radiotherapy

BACKGROUND The optimal sequence and extent of multimodality therapy remains to be defined for extrapulmonary small cell carcinoma because of its rarity. The purpose of our study was to assess the response to neoadjuvant chemotherapy followed by chemoradiation/radiation in patients with extrapulmonary small cell carcinoma. MATERIALS AND METHODS Four consecutively diagnosed patients were included in this study. The primary tumor site was oropharynx in three patients and esophagus in one. The patients with the limited disease were treated with chemotherapy followed by concurrent chemoradiation (n=2) or radiotherapy (n=1). The patient with the extensive disease with the primary site in vallecula was treated with chemotherapy and palliative radiotherapy to the metastatic site. RESULTS The median follow-up was 22.5 months (range, 8-24 months). Three patients with the limited disease (base of tongue, n=2; esophagus, n=1) were in complete remission. The patient with the extensive disease died of loco-regional tumor progression at 8 months from the time of diagnosis. CONCLUSIONS The combination of chemotherapy and radiotherapy is the preferred therapeutic approach for patients with extrapulmonary small cell carcinoma. Induction chemotherapy followed by concurrent chemoradiation or radiation provides a good loco-regional control in patients with limited disease.

Immunohistochemical characterization of glandular elements in glandular cardiac myxoma: Study of six cases

Back ground: Glandular cardiac myxoma has varying clinical presentation with uncertain histogenesis and debatable immunohistochemical profile. Glandular epithelial differentiations are rare phenomenon known to be present as an intrinsic component of the tumor. The origin of the glands has been attributed to epithelial differentiation of a totipotent cardiomyogenic precursor cells or the entrapped foregut rests in the tumor. Materials and Methods: Retrospective study includes six cases of glandular cardiac myxoma collected over a perior of 4 years. Sections were examined to define the histogenesis, histological and immunohistochemical profile of the glandular elements. Results: Incidence of glandular cardiac myxoma was 6.6% with a male to female ratio of 1:2.Mean age was 49.9 years. Left atrium was the commonest site. Five were sporadic and one was familial. Chest pain and dyspnea were the commonest clinical symptoms. Histologically all myxoma showed well formed glandular structures with typical myxomatous area. No atypia, mitosis or necrosis was identified in the glandular elements. Markers in six cases of glandular cardiac myxoma were immunopositive for CK7, CK 19, EMA, CEA, focally for E-cadherin while immunonegative for CK20, Chromogranin, Synaptophysin, calretenin, vimentin, B-catenin, TTF-1 and GCDFP-15 favoring enteric differentiation. Conclusion: Glandular cardiac myxoma is a rare entity which shows characteristics similar to those of classical cardiac myxoma with benign glandular elements showing enteric differentiation. Complete surgical excision is the treatment of choice with good prognosis. It is important to recognize this entity to avoid an erroneous diagnosis of metastatic adenocarcinoma.

Synchronous existence of granular cell tumor and small cell carcinoma of lung: An unusual entity

Granular cell tumor (GCT) is a rare benign mesenchymal tumor that uncommonly occurs in the lung and tracheobronchial tree. Small cell carcinoma of lung is a centrally located malignant neoplasm that commonly occurs in elderly smokers. Concomitant existence of both the neoplasm in lung is extremely rare with only one reported case in the literature. Few rare combinations of GCT with other primary bronchogenic carcinomas have also been reported. Clinical symptoms depend upon the site and size of the tumor. Definitive diagnosis is by histopathological and proper immunohistochemical analysis. Identification of this entity is important as treatment requires individual therapy protocols that depend on the presence of metastasis, location of the tumors, and type of bronchogenic carcinoma.

Malignant Peripheral Nerve Sheath Tumour of the Maxilla

A 38-year-old man was diagnosed with malignant peripheral nerve sheath tumour of the maxilla. He was treated with total maxillectomy. Histopathological examination of the resected specimen revealed a close resection margin. The tumour was of high grade with an MIB-1 labelling index of almost 60%. At six weeks following the surgery, he developed local tumour relapse. The patient succumbed to the disease at five months from the time of diagnosis. The present report underlines the locally aggressive nature of malignant peripheral nerve sheath tumour of the maxilla which necessitates an early therapeutic intervention. A complete resection with clear margins is the most important prognostic factor for malignant peripheral nerve sheath tumour in the head and neck region. Adjuvant radiotherapy may be considered to improve the local control. Future research may demarcate the role of targeted therapy for patients with malignant peripheral nerve sheath tumour.

Disseminated Aspergillus flavus following septic arthritis in an immunocompetent patient: a case report

BackgroundAspergillosis is a rare cause of osteomyelitis and septic arthritis. Aspergillus osteomyelitis is a debilitating infection affecting both immunocompromised and immunocompetent patients. It is associated with a high incidence of morbidity and mortality. Infection with Aspergillus flavus species in the knee has been very rarely seen in the past.Case presentationWe present a case of septic arthritis of the knee in an Indian patient secondary to infection with Aspergillus flavus, which was earlier managed as a case of tuberculosis based on the endemicity of the condition, later leading to disseminated aspergillosis. There was no clinical feature or investigation suggesting immunocompromised state. Following knee arthrotomy, Aspergillus flavus was isolated and patient was subsequently managed with antifungals leading to recovery after three months.ConclusionsDisseminated aspergillosis can mimic tuberculosis both clinically and radiologically. Though fungal infections affect joints rarely but they must always be ruled out to avoid later complications.

Nora’s lesion of 2nd metacarpal treated by wide excision, autologous fibular grafting and metacarpophalangeal joint replacement: A rare case report☆

Bizarre paroxysmal osteochondromatous proliferation (BPOP) is a rare benign neoplastic lesion predominantly affecting small bones of the hand and known for its recurrence after local excision. We describe a rare case of BPOP involving second metacarpal of the dominant hand which was treated using a novel technique. Wide excision and reconstruction with non-vascularised fibular autograft was performed along with metacarpophalangeal joint replacement. At a follow up of two years, there was no evidence of recurrence and patient had good functional outcome. Hence this modality of treatment could be considered in dealing such cases of BPOP involving the small bones of hand.

Familial biatrial cardiac myxoma with glandular elements: A Rare entity with review of literature

Cardiac myxomas are benign neoplasm of the heart with an incidence of 0.3%. Glandular cardiac myxomas are very rare and accounts for less than 3% of all cardiac myxomas. Here, we report a case of familial glandular cardiac myxoma in a 35 year old male who complained of exertional dyspneoa and weakness of right side of body on clinical presentation. Associated features of Carneys complex were not present. Family history revealed presence of cardiac myxoma in younger brother and sister. Transthoracic echocardiography detected biatrial myxoma. Excision of both lesions was done under cardiopulmonary bypass. Histopathology confirmed myxoma with glandular elements. Postoperative course was uneventful.

Vascular Cyst of Adrenal: A Report of Two Cases

Vascular cysts of the adrenal gland are rare heterogeneous group of lesions with an autopsy incidence of 0.06%. They have female preponderance with most common presentation being pain abdomen. Cysts of the adrenal glands are classified as (a) parasitic cysts (b) epithelial cyst (c) pseudocyst and (d) endothelial cysts. Out of these endothelial cysts constitute about 84% of the cases. As the clinical features and radiological findings of these lesions are nonspecific, a proper histological examination along with immunohistochemistry are necessary for a definitive diagnosis. Here, we report two cases of vascular endothelial cyst of the adrenal gland to emphasize the histomorphological and immunohistochemical features of these lesions.

Sclerosing liposarcoma of the anterior mediastinum: An unusual case.

Liposarcomas are extremely rare in the mediastinum. Patients usually present late due to the compressive effect of the tumor on the adjacent structures. Severity of the symptoms depend mainly on the size of the tumor and the structure it infiltrates. Well differentiated slow growing liposarcomas are the most common ones in the mediastinum followed by dedifferentiated and poorly differentiated ones. These tumors have bad prognosis because of incomplete surgical excision due to its inaccessible location. Hence these patients should be kept under close follow up because of high recurrent rates. Here we are presenting a rare case of anterior mediastinal sclerosing liposarcoma in a 77 year old male.