Rare case of extraskeletal Ewings sarcoma of the sinonasal tract

Ewings sarcoma (ES) and primitive neuroectodermal tumor are closely related family of small round cell tumors seen in childhood and adolescence. The incidence of these tumors occurring in the head and neck region is just 2-7%. Mandible and maxilla are the most common sites, whereas involvement of the sinonasal tract is very rare. We report a case of extraskeletal ES of the sinonasal tract in a 29-year-old female who presented with nasal obstruction and epistaxis. The patient was treated with 14 cycles of chemotherapy, combined with surgery and radiotherapy with complete recovery. We present this case due to its rarity, to analyze the clinical, histopathological and immunohistochemical findings, so as to differentiate from other small round cell tumors of the sinonasal tract for appropriate treatment.     

A rare case of primary bone xanthoma of the clivus

Primary bone xanthoma is a rare type of tumor, and those developing primarily within the skull are even more unusual. In this case, a primary bone xanthoma of the clivus without endocrine or metabolic complications represents the first of this type to be reported. The patient, a 24-year-old woman, initially experienced frequent headaches. Subsequent skull tomography and bone-window computed tomography (CT) revealed a clearly demarcated osteolytic lesion in the clivus. T1-weighted magnetic resonance imaging (MRI) exhibited low intensity, and T2-weighted MRI showed iso-high intensity and a heterogeneously faint contrast effect. The tumor was completely resected, after which the patient’s headaches disappeared completely. Because xanthoma is devoid of neoplastic features, it does not necessarily require aggressive therapy. Therefore, it is very important to understand the characteristics of its clinical symptoms and to give due consideration to differential diagnosis based on pathological presentations and imaging features. This study introduces information regarding a bone xanthoma originating within the skull, together with a review of bone xanthoma literature.     

A rare case of nasal desmoid tumor

Desmoid tumor or Aggressive Fibromatosis is rarely seen in the nasal cavity and are prone to recurrences. A patient with earlier history of excision was treated with repeat ergery employing the Weber-Ferguson incision followed by post-op radiotherapy.     

A rare case of primary nasoethmoidal meningioma

Meningiomas of sinonasal tract is a rare entity especially in children. Primary sinonasal meningiomas often pose difficulty in diagnosis because of their infrequent occurrence and clinically they appear to be nasal polyp. The final diagnosis rests on the histological examination. Extra cranial meningiomas are rare in children and tend to be more aggressive. Here a case of primary ethmoid sinus meningoma with extension into the nasal cavity is presented and despite of its aggressive behavior it was benign. The importance of complete surgical extirpapion is undoubted and results in a good survival rate. Histological grading of the tumour is not crucial in predicting the rate of recurrence. The paucity of reported cases is the evidence for its rarity.     

A rare case of male primary breast lymphoma

Primary lymphoma of the breast is a rare occurrence because of the paucity of lymphoid tissue in the breast and is an even rarer entity in the male breast. Imaging, along with tissue diagnosis goes a long way in diagnosing breast lymphoma which has a significantly different management scheme than other breast neoplasms with respect to radio-chemotherapy rather than surgical resection. We present a case of primary male breast lymphoma which was evaluated with magnetic resonance imaging as well as other conventional imaging modalities and was treated by chemotherapy with a 7-month follow-up.     

A rare case of primary extracranial meningioma of the Paranasal Sinuses

A rare case of Extracranial Meningioma with Paranasal Sinus involvement has been reported with review of available literature.     

Importance of studying primitive neuroectodermal tumors and extraosseous Ewings sarcoma of the vagina and vulva

Primitive neuroectodermal tumor (PNT) and Ewing''s sarcoma are rare, round-cell tumors, characterized by the presence of the t(11; 22)(q24; q12) chromosomal translocation. A review of the literature revealed only 38 previously reported cases of vulvar PNT and Ewing''s sarcoma and 15 vaginal PNT and Ewing''s sarcoma. Although rare, these types of tumors should be taken into consideration when making a differential diagnosis for vulvar or vaginal tumors. The currently available data is limited, and therefore, case reports are essential for improving knowledge and management of these types of extremely rare tumors. However, further molecular and histopathological studies are essential for an improved understanding of these conditions and for an early, correct diagnosis. Although the gathered and presented data from the present review are limited, the literature demonstrates that the outcome of these types of cancer are more favorable compared with outcomes observed for carcinomas in more typical locations.     

Primary breast synovial sarcoma: a rare primary breast neoplasm

A primary synovial sarcoma based on the breast is rare. The usual tumours on the breast are carcinomas. Synovial sarcomas account for about 6–9% of soft tissue sarcomas and most commonly develop in the extremity of young adults (80%). The other 20% of synovial sarcomas can arise in non-extremity sites (trunk 8%, retroperitoneal/abdominal 7%, head and neck 5%) but synovial sarcomas can develop in almost any other anatomical location. We report a case of a young woman who presented with a suspected common breast tumour and started treatment of this tumour with carcinoma neoadjuvant chemotherapy. We were surprised when the pathologist identified a synovial sarcoma in the histopathology study.     

Pleomorphic liposarcoma of bone: a rare primary malignant bone tumour

Background

Papillary intralymphatic angioendothelioma (PILA) is a locally aggressive, rarely metastasizing vascular tumor, generally occurring in the soft tissues, with less than 40 cases described in the literature and only three cases reported in bone.

Case presentation

We describe the case of a 51-year-old male with an intraosseous PILA of the proximal edge of his left clavicle and two other lesions evident on imaging. The patient was treated with marginal resection of the clavicle lesion but was lost to follow-up 1 month after surgery.

Conclusions

PILA can also occur in bone, albeit very rarely, and has to be considered in the differential diagnosis of vascular bone tumors.

     

A rare case of intravascular lymphoma diagnosed on bone marrow trephine

Intravascular lymphoma (IVL) is an extremely rare form of extra-nodal non-Hodgkins lymphoma characterised by the proliferation of neoplastic lymphocytes within the lumina of small arteries, veins and capillaries. The great majority of reported cases appear to be of B cell lineage. There is a wide variation in clinical presentation, and multiple organs are usually affected. We report a case of a 67-year-old man who presented with constitutional symptoms and neurological deficit and was diagnosed following bone-marrow trephine. His disease responded to polychemotherapy treatment but he died 15 months after diagnosis. This case in unusual in that it is generally felt that bone marrow is relatively spared until late in the disease and is often not clearly demonstrable histologically. In addition, this case supports the limited data that responses can be obtained following polychemotherapy treatment, although the prognosis remains generally poor.     

A rare case of primary gastric plasmacytoma: an unforeseen surprise

Primary plasmacytoma of the gastrointestinal tract is a rare entity. We report a case of a primary gastric plasmacytoma in a 57-year-old man who presented with upper-gastrointestinal bleeding. Endoscopy showed a nodular gastric mass with central umblication. Histological examination of the gastrectomy specimen revealed a monoclonal lambda-chain extramedullary plasmacytoma. Further staging was found to be negative for multiple myeloma. As other more common pathologic processes at this site may also be endowed with numerous plasma cells, awareness of this entity and distinction using immunohistochemistry are extremely crucial. Because systemic disease ultimately develops in many patients with localized plasmacytoma, such patients should be followed closely for the appearance of clinical, biochemical, and roentgenologic evidence of multiple myeloma.     

Thorotrast-associated sarcoma of bone: A case report and review of the literature.

An osteosarcoma developed near the right lesser trochanter of a 55-year-old woman. The neoplasm fulfilled the generally accepted criteria for a Thorotrast related malignancy. Strengthening this relationship was the occurrence of the tumor in an unusual location and uncommon age group. Thorium was confirmed both in the tumor and in bone marrow histiocytes by its characteristic x-ray spectrum. Including this case only twelve Thorotrast-associated neoplasms of bone have been reported. The mean latency period after Thorotrast administration was 26 years. Regression analysis revealed that latency period and Thorotrast dose are inversely related. All tumors were reported to be sarcomas. Six osteosarcomas, one fibrosarcoma, one chondrosarcoma, and one undifferentiated sarcoma were proven histologically. All patients, for whom follow-up was given, had died of the tumor. The reasons behind the low frequency of reported Thorotrast-associated bone malignancies may be the low concentration of 232Th and radioactive daughters in bone, long latency period or the general lack of knowledge concerning the possible relationship between Thorotrast and bone tumors. Statistical association between Thorotrast injection and bone sarcoma is shown. The criteria for the diagnosis of Thorotrastosis are discussed as well as the long term deleterious effects. The distribution of Thorotrast within the body is discussed as well as the associated radiographic changes.