Multifocal synchronous osteosarcoma

July 9, 2017 | Autor: Massimiliano Oliveri | Categoria: Humans, Osteosarcoma, Male, Clinical Sciences
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European Journal of Radiology 20 (1995) 43-45

Multifocal synchronous osteosarcoma A. Taccone a, M. Di Stadio b, M. Oliveri a, M. Oddone a, M.Occhi *a aDepartment of Radiology, Gaslini Children's Hospital, Largo G Gaslini 5, 16148 Genoa, Italy bDepartment of Orthopaedics, Gaslini Children's Hospital. Genoa, Italy

Received 27 January 1995; revision received 13 March 1995; accepted 16 March 1995

Keywords: Neoplasm; Bone; Osteosarcoma, multicentric

I. Introduction Multifocal osteosarcoma or osteosarcomatosis is a rare pathological condition, characterized by two or more foci of osteosarcoma in the skeleton, apparent at the time of presentation, with or without pulmonary metastases [1-3]. Whether or not this tumor represents a true multifocal osteosarcoma or an aggressive metastatic spread of a unifocal osteosarcoma remains a controversial point [2]. The patient presented here best fits the description of multicentric osteosarcoma. 2. Case report A 5-year-old boy presented with a 3-week history of limping and left thigh pain following a fall. On physical examination a slight swelling was noted. Hypotrophia of the crural muscles and coxofemural joint stiffness were also present. Radiographs of the left femur and knee showed a dramatic overall involvement of this segment by a large, sclerotic neoplastic process, with cortical disruption, periosteal reaction and tumor extension into adjacent soft tissues (Fig. la). Secondary asymptomatic foci were discovered in the proximal metaphyses of the left and right tibia (Fig. lb), in the proximal metaphysis of the right humerus and in the lungs. Computed tomography best demonstrated calcified pulmonary metastases (Fig. 2); detecting a greater num-

* Corresponding author, Tel.: +39 10 5636529; Fax: +39 10 37776590.

ber of lesions than on plain films, in lungs and mediastinum. Laboratory findings revealed increased serum LDH activity and elevated levels of alkaline phosphatase. A biopsy from the left middle femur showed a high grade malignant neoplasm that filled the marrow spaces. The cells were producing new bone and osteoid matrix; mitotic activity was abundant. Monomorphous and round cells were noted focally. The patient was immediately started on chemotherapy which included Vincristine, Doxorubicine and Cisplatin. Despite therapy, radiographic evaluation demonstrated enlargement of the lesions and increasing radiodensity. A second course of chemotherapy was given, but the lesions continued to grow. Further treatment was refused by the parents. 3. Discussion Multifocal osteosarcoma continues to present a diagnostic and therapeutic challenge: the treatment of children with common malignant solid tumors has become increasingly successful in the past three decades, but these patients still have a bad prognosis. The ongoing debate as to whether or not this tumor is a true multifocal skeletal disease or an aggressive metastatic pattern of osteosarcoma, remains unresolved. The 'metastatic' theory, proposed by several authors, is based on clinical radiological considerations such as the presence of a dominant lesion, larger in size and more aggressive. Proponents of the other hypothesis describe multiple, synchronous osteosarcomas, arising from symmetrical regions of the skeleton; the multifocality is suggested by the absence of lung disease in

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A. Taccone et al./ European Journal of Radiology 20 (1995) 43-45

a

b

Fig. 1. (a) Radiograph of the left femur showing large sclerotic lesions involving distal and proximal metaphyses with extension to the diaphysis. Cortical disruption and periostal reaction are also present. Similar lesions are present in the lower right femur. (b) An anteroposterior (AP) film of the tibias shows the extension of the disease to juxtametaphyseal regions, containing patchy areas of ossification.

Fig. 2. Multiple diffuse sclerotic metastases were identified in an AP view of the chest. Use of chest CT scan (here) resulted in a higher percentage of detected lung nodules, a better definition of their calcified structure and detection of mediastinal adenopathies.

some o f these patients. F u r t h e r m o r e , some a u t h o r s advanced the theory that o s t e o s a r c o m a t o s i s arose from widespread areas o f b o n e dysplasia, with d e v e l o p m e n t o f simultaneous osteoblastic t u m o r s [2,4]. Conversely, in favor o f the metastatic theory, the vertebral venous plexus system has been recognized as an a n a t o m i c a l bypass for metastatic spread to bones, without involvement o f the lungs [2]. F u r t h e r m o r e , no unusual pathological features have been described: so far, m a n y cases have been r e p o r t e d since it was first described, but histologically, it resembles o s t e o s a r c o m a [1,2,5-7]. Serum markers, L D H a n d alkaline p h o s p h a t a s e as in osteosarcoma, can be used as a disease m o n i t o r and their levels increase during t u m o r growth. R a d i o g r a p h i c a l l y , the multiple foci have features o f typical o s t e o s a r c o m a [2]. In o u r case also, we found symmetric and simultaneously a p p e a r i n g skeletal foci with a metaphyseal distribution a n d p u l m o n a r y secondary calcific lesions, b o t h p o o r responders to chemother-

A. Taccone et al. /European Journal of Radiology 20 (1995) 43-45

apy: 2 m o n t h s after the diagnosis, a slight disease progression was documented. The perspectives for u n d e r s t a n d i n g the biology o f this neoplasm a n d i m p r o v i n g the outcome of affected patients, are based on molecular biology a n d genetic studies of m u l t i p r i m a r y osteosarcoma cell lines [8]. Previous reports have shown that these patients have a very high incidence o f both somatic a n d germ-line p53 m u t a tions, thus strongly implicating this gene in the pathogenesis of multifocal osteosarcoma [9].

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