Primary osteoliposarcoma of bone

Cremer, H; Koischwitz, D; Tismer, R

doi:10.1007/bf00413314

Cited by 14 publications

(6 citation statements)

References 6 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…They account for 10 to 15% of soft-tissue sarcomas. 5 Primary liposarcomas of the bone, arising from lipoblasts in the fatty bone marrow, are extremely rare and constitute <0.1% of primary bone tumours, 6 with a wide anatomic distribution (including the femur, [7][8][9][10][11][12][13] humerus, 3,14-17 tibia, 6,18-20 fibula, 21,22 ulna, 23 spine, 24,25 ilium, 10 scapula, 26 and skull [27][28][29] ). Less than 35 cases have been reported, 9 and only 20 of these fulfilled the criteria for primary liposarcoma of bone, 2 and only 16 had definitive photomicrographs.…”

Section: Discussionmentioning

confidence: 99%

“…Five-year survival rates are <67%, with up to 50% of patients developing local recurrence or metastatic disease within 5 years, 7,17,[30][31][32][33][34] and tumours may recur even 10 years after treatment. 35 Primary liposarcomas of the bone appear to follow a course similar to that of the soft-tissue liposarcomas, 10 and have a more favourable prognosis than osteosarcomas.…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Primary Intra-Osseous Liposarcoma of the Femur: A Case Report

Macmull¹,

Atkinson²,

Saso³

et al. 2009

J Orthop Surg (Hong Kong)

View full text Add to dashboard Cite

We report a rare case of an intra-osseous liposarcoma of the proximal femur. A 26-year-old man presented with a 6-month history of left groin pain radiating to the knee and an antalgic gait. Radiology showed a predominantly fatty lesion in the medial aspect of the femoral neck extending toward the lesser trochanter; most of the marrow in the femoral neck had been replaced without evidence of an extra-osseous mass; and the posterior cortex had been destroyed. Histological and immunohistochemical analyses of the tumour after open biopsy were indicative of highgrade liposarcomatous malignancy. After exclusion of any other primary tumour foci or metastases on regional and whole-body magnetic resonance images, the diagnosis of a high-grade intra-osseous primary liposarcoma of the proximal femur was made. The patient received 2 preoperative courses of neoadjuvant doxorubicin, cisplatin and methotrexate. After proximal femoral replacement following en bloc excision of the proximal femur, 4 more cycles of adjuvant ifosfamide and etoposide were given. At the 16-month follow-up, he remained independently ambulatory, with no local or distant recurrence. Tissue diagnosis and multimodal imaging, rather than any single radiological investigation, are important in making the diagnosis.

show abstract

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Primary Intra-Osseous Liposarcoma of the Femur: A Case Report

Macmull¹,

Atkinson²,

Saso³

et al. 2009

J Orthop Surg (Hong Kong)

View full text Add to dashboard Cite

show abstract

“…Primary malignant mesenchymoma of bone is distinctly rare, and to the best our knowledge, only 15 cases have been described in the English literature (4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16). The details of these reported cases, in chronological order, together with those of our patient, are summarized in Table 1.…”

Section: Discussionmentioning

confidence: 99%

“…The vast majority of cases of malignant mesenchymoma occur in the soft tissue, and primary malignant mesenchymoma of bone is extremely rare. The first such case was described by Lichtenstein in 1952 (4), and thereafter only around 14 cases have been reported in the English literature (5–16). In this article, we report a case of malignant mesenchymoma, comprising osteosarcoma, chondrosarcoma and rhabdomyosarcoma, of the fibula in a 10‐year‐old female student.…”

mentioning

confidence: 99%

Primary osteochondrorhabdomyosarcoma (malignant mesenchymoma) of the fibula: a rare tumour in an unusual site - Case report and review of the literature. Case report

Chow¹,

Kumta

2004

Apmis

View full text Add to dashboard Cite

Malignant mesenchymoma, defined by Stout as sarcomas comprising two or more unrelated differentiated tissue elements other than a fibrosarcoma component, is rare. We report a case of primary malignant mesenchymoma of the proximal fibula in a 10-year-old female student who presented with pain and swelling of the right knee for 2 months. Initial biopsy showed features of rhabdomyosarcoma only, but the resected specimen revealed additional osteosarcomatous and chondrosarcomatous elements. The patient remained well more than 5 years after initial presentation. Including our present patient, 16 cases of primary malignant mesenchymoma of bone are found in the English literature, affecting mainly adolescents and young adults, with a slight male predominance and predilection for the metaphysis of long bones, especially around the knee. More than 60% of the patients develop metastasis, almost invariably to the lung, but occasionally to the brain. About 60% of the patients, all with metastasis, died mostly within one year of diagnosis. The clinical features of primary malignant mesenchymoma of bone thus resemble those of conventional osteosarcoma. Moreover, our case illustrates that, with combination chemotherapy targeted for individual elements, the prognosis of this rare tumour might be much improved, as in osteosarcoma.

show abstract

“…Malignant mesenchymoma arising in bone is exceedingly rare. The first example was described by Lichtenstein in 1952 (8), and thereafter only around 20 cases have been reported in the English literature, all as single or two case reports (9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19)(20)(21)(22)(23)(24)(25)(26). For better characterization of this tumor and in accordance with recent terminology employed for the designation of neural tumor showing divergent differentiation of neurofibroma, schwannoma and/ or perineurioma as 'hybrid nerve sheath tumor' (27)(28)(29), it is proposed for reasons detailed in our discussion that malignant mesenchymoma should be renamed as 'composite sarcoma'.…”

mentioning

confidence: 99%

Critical reappraisal of primary osseous composite sarcoma (malignant mesenchymoma) – analysis of four cases and literature review

Chow

2016

APMIS

View full text Add to dashboard Cite

In accordance with recent terminology, it is proposed that malignant mesenchymoma should be renamed 'composite sarcoma' and defined as 'a sarcoma composed of two or more cellular types each of which is sufficiently differentiated to permit clear recognition of its histogenetic type microscopically, immunohistochemically or ultrastructurally; excluding fibrosarcomatous and high-grade pleomorphic undifferentiated sarcomatous component, dedifferentiated sarcoma and the combination of osteosarcoma and chondrosarcoma which is regarded as a single histogenetic type'. Four cases of primary osseous composite sarcoma (POCS) were identified among 928 primary bone sarcomas. Their age ranged from 10 to 87 years, peak incidence in the second decade with equal sex distribution. Most presented with pain, commonest in the knee, affecting the metaphysis, appearing radiologically as expansile infiltrative osteolytic lesions with cortical erosion, periosteal reaction, variable extent of osteoblastic areas and soft tissue extension. All contained variable amounts of conventional high-grade osteosarcoma with or without chondrosarcoma component; the other constituents were liposarcoma, rhabdomyosarcoma and leiomyosarcoma. In all cases, Ki67 proliferative index was over 35%, there was no CDK4 and MDM2 amplification. The absence of low-grade component supported the de novo origin of POCS rather than derivation from divergent dedifferentiation. The two older patients with hitherto undescribed osteoleiomyosarcoma died 2 and 10 months after operation, whereas the two younger with osteorhabdomyosarcoma and osteoliposarcoma enjoyed disease-free survival at 16 and 6 years after chemotherapy despite the latter showing lung metastasis at presentation. Identification of the different lines of differentiation together with their approximate amounts and histological grades is therefore mandatory for POCS as multi-agent chemotherapy catered for each sarcoma component might offer hope for long-term disease-free survival.

show abstract

Primary osteoliposarcoma of bone

Cited by 14 publications

References 6 publications

Primary Intra-Osseous Liposarcoma of the Femur: A Case Report

Primary Intra-Osseous Liposarcoma of the Femur: A Case Report

Primary osteochondrorhabdomyosarcoma (malignant mesenchymoma) of the fibula: a rare tumour in an unusual site - Case report and review of the literature. Case report

Critical reappraisal of primary osseous composite sarcoma (malignant mesenchymoma) – analysis of four cases and literature review

Contact Info

Product

Resources

About