Primary retroperitoneal extraosseous osteogenic sarcoma: A case report and review of the literature.

A case of retroperitoneal extra-osseous osteogenic sarcoma is reviewed, as well as the criteria necessary for the diagnosis. The general characteristics and the differential diagnosis of the tumor are also presented.     

Primary osteogenic sarcoma of the clavicle. A case report

The clavicle is an unusual site for any primary bone tumor, including osteogenic sarcoma. A paucity of data in the literature on this type of lesion makes clear understanding of the optimal treatment difficult. Given its anatomical location, the lesion requires a multidisciplinary approach for optimal care, with each member of the health care team being important for maintaining and prolonging the quality of life for a patient. A 22-year-old man with primary osteogenic sarcoma of the clavicle was treated with preoperative and postoperative chemotherapy and was managed with surgical excision of the tumor while sparing the limb. Clinically disease-free for 20 months postoperatively, the patient presented with gross evidence of local recurrence with diffuse metastatic involvement at 22 months and died 6 weeks later. We have found no report of long-term survival on a patient with this lesion.     

Extraosseous osteogenic sarcoma arising from the retroperitoneum: A case report

A 44-year-old housewife with a mass in the left upper quadrant of the abdomen was admitted. The tentative diagnosis was cystadenocarcinoma arising from the pancreatic tail, as determined by CT and selective angiography. Excision of the mass with removal of the spleen, pancreatic tail, left renal capsule and surrounding lymphnodes was performed. Photomicroscopic sections revealed proliferation of malignant cells and malignant osteoids. The final diagnosis was osteogenic sarcoma arising from the retroperitoneum. among the extraosseous osteogenic sarcoma arising from the somatic soft tissue, involvement of the retroperitoneum is rare. Only 12 cases including our own case have been reported as being of retroperitoneal origin. No patient survived for five years and in our patient, there are signs of recurrence one year after the surgery. A part of this study was reported at the 135th meeting of the Kinki Surgical Society on April 28, 1984 at Nishinomiya, Hyogo, Japan     

Human osteogenic sarcoma

Summary The morphology of 26 cases of osteogenic sarcoma was studied using electron microscopic techniques, and the localization of acid and alkaline phosphatase activity at the ultrastructural level elucidated. Four different cells were present in the tumours: osteoblast-like, fibroblast-like, chondroblast-like, and multinucleated giant cells. The osteoblast-like cell was present in most of the tumours studied. Acid phosphatase activity was present in lysosome-like structures of almost all the cell-types studied. Alkaline phosphatase activity was noted in or on the plasma membranes and associated vesicles of osteoblast-like, fibroblast-like, and multinucleated giant cells. The abundant reaction product deposition of alkaline phosphatase as compared with the lower acid phosphatase activity is in agreement with the nature of this bone-forming tumour. The results of the histochemical studies have added to the understanding of the pathobiology of the different cells composing osteogenic sarcomas.

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Résumé La morphologie de 26 sarcomes ostéogéniques a été étudiée par microscopie électronique et la localisation des phosphatases acide et alcaline a pu être précisée au niveau ultrastructural. Quatre différents types de cellules ont été mis en évidence dans les tumeurs: cellules d'aspect ostéoblastique, fibroblastique et chondroblastique, ainsi que des cellules géantes multinuclées. Les cellules d'aspect ostéoblastique étaient présentes dans presque toutes les tumeurs étudiées. Une activité phosphatasique acide a été trouvée dans les lysosomes de toutes les cellules étudiées. Une activité phosphatasique alcaline a été observée dans — ou sur — la membrane plasmatique et dans les vésicules associées des cellules d'aspect ostéoblastique et fibroblastique ainsi que des cellules géantes. L'abondance des produits de réaction de la phosphatase alcaline contrastant avec une faible activité phosphatasique acide est en accord avec la nature de cette tumeur ostéogénique. Les résultats de l'étude histochimique ont aidé à comprendre la pathobiologie des différentes cellules qui constituent les sarcomes ostéogéniques.

     

Giant extraosseous Ewing sarcoma of the lung in a young adolescent female--a case report

Extraosseous Ewing sarcoma is a rare soft tissue tumour that is histologically indistinguishable from the bone Ewing sarcoma. The translocation involving chromosome 22 along with CD 99 expression is pathognomonic and is useful in differentiating from other small round cell tumours. Primary lung involvement by this malignant tumour is very uncommon and up to this date only ten cases have been reported. We report a further case in a 15 year-old-female who presented with a huge lung mass causing an opaque haemithorax.     

Primary intradural extraosseous Ewing sarcoma of the spine: case report and literature review

BACKGROUND AND IMPORTANCE: To report a rare case of spinal intradural extraosseous Ewing sarcoma in an adult and review current literature. Although Ewing sarcoma belongs to the family, the treatment modalities are different, and thus the correct diagnosis is very important despite its rare occurrence. CLINICAL PRESENTATION: A 56-year-old woman presented with nocturnal bilateral buttock and leg pain. Magnetic resonance imaging (MRI) showed an enhancing intradural extramedullary extraosseous tumor at L1. INTERVENTION: A T12-L2 laminectomy was performed to resect the tumor. Immunohistochemical analysis confirmed the diagnosis of Ewing sarcoma. A thorough diagnostic workup did not reveal any bony origin of the tumor. Primary intradural central nervous system Ewing sarcoma is infrequently encountered and shares imaging and histopathological features with central primitive neuroectodermal tumors. Establishment of the right diagnosis is crucial because it mandates a distinct workup and treatment modality different from that for central primitive neuroectodermal tumor. Although osseous Ewing sarcoma predominantly occurs in children and young adults, extraosseous central nervous system Ewing sarcoma is not uncommon in adults and should therefore be considered in the differential diagnosis of extraosseous small blue cell tumors in adult patients.