scholarly journals Proximal epithelioid sarcomatous dedifferentiation in secondary chondrosarcoma in a known case of multiple osteochondromatosis

2017 ◽  
Vol 9 (03) ◽  
pp. 214-216
Author(s):  
Biswajit Dey ◽  
Mangesh Chikhale ◽  
Prita Pradhan ◽  
Adarsh Barwad ◽  
Bhawana Ashok Badhe

AbstractOsteochondroma is the most common benign bone tumor. Approximately 15% of osteochondromas occur as multiple lesions. Multiple osteochondromatosis has a higher risk of developing chondrosarcomas, which are of low grade with good prognosis. About 10% of all chondrosarcomas may undergo dedifferentiated change, which has a poorer prognosis. Dedifferentiated peripheral chondrosarcoma developing within an osteochondroma is extremely rare. Dedifferentiation usually occurs in the form of osteosarcoma, malignant fibrous histiocytoma, fibrosarcoma, or rhabdomyosarcoma. We report a case of proximal epithelioid sarcomatous dedifferentiation in secondary chondrosarcoma in a 39-year-old male with multiple osteochondromatosis in bilateral arm. To the best of our knowledge, epithelioid sarcomatous dedifferentiation has not been described in the literature.

2020 ◽  
Vol 38 (15_suppl) ◽  
pp. 11525-11525
Author(s):  
Lina Tang ◽  
Xiaohui Niu ◽  
Zhen Wang ◽  
Qiqing Cai ◽  
Chongqi Tu ◽  
...  

11525 Background: Primary malignant bone tumors are rare forms of cancer and include mainly bone sarcomas, which are categorized into 3 common types based on tissue origin: osteosarcoma, chondrosarcoma and Ewing sarcoma. A phase II trial was designed to explore the anlotinib activity in patients with relapsed or metastatic primary malignant bone tumor. Methods: Eligible pts were received 12mg of anlotinib once daily, 2 weeks on and 1 week off until progression or unacceptable toxicity. Key-eligibility criteria were aged 14-70 years, histologically confirmed diagnosis of osteosarcoma, chondrosarcoma, bone derived malignant fibrous histiocytoma, giant cell tumor, Ewing sarcoma and PNET, confirmed previous chemotherapy failure, ECOG 0-1(0-2 for amputation pts), required at least one measurable lesion. We observed PFS, OS, ORR, DCR and AE in this study. Results: From August 2018 to April 2019, 42 pts were included. Of 42 efficacy-evaluable pts, 25 were man, median age was 28 (14-68) years. There were 29 pts of osteosarcoma, 9 pts of chondrosarcoma, 3pts of Ewing sarcoma and 1 pt of bone derived malignant fibrous histiocytoma. The progression-free rate at 12 weeks (PFR12weeks), ORR and DCR were 71.3%, 9.52% and 78.57%. Median PFS was 5.26 months (95%CI = 3.48-8.44). Median OS was 11.40 months (95%CI = 10.09, [ ). Median PFS of osteosarcoma and chondrosarcoma was 4.83 months (95%CI = 3.48, 7.13 ) and 2.76 months (95%CI = 1.31, [ ) respectively. The most common Gr 3-5 anlotinib-related AEs were hypertension (19.05%), hypertriglyceridemia (9.52%), hand-foot syndrome (7.14%), and proteinuria (4.76%). Conclusions: The phase II study shows a promising activity of anlotinib in patients with relapsed or metastatic primary malignant bone tumor and an acceptable toxicity. Clinical trial information: NCT03527888 .


HPB Surgery ◽  
1989 ◽  
Vol 1 (3) ◽  
pp. 233-237 ◽  
Author(s):  
J. F. W. Garvey ◽  
A. Ng ◽  
J. F. England ◽  
D. M. Sheldon

A case of fibrous histiocytoma of low grade malignancy arising from the uncinate lobe of the pancreas is reported. This is an unusual site for these extremely rare tumours. Survival up to 4 years has been achieved in our patient following surgical resection.


2018 ◽  
Vol 1 (Supplement) ◽  
pp. 44
Author(s):  
A.M. Bratu ◽  
I.A. Sălcianu ◽  
A.I. Nicula ◽  
C. Zaharia ◽  
A.N. Marinescu

Abstract Giant cell tumor of soft tissue (GCTST) is usually of synovial origin. It affects synovial membrane, serous bursae, and tendinous tunnels. The most common localizations are in the hands and forearms. Anatomopathological, GCTST is considered as being composed of a cellular fibroblastic stroma in which the tumor cells are distributed. This type of tumor is composed of a mononuclear complex and osteoclast-like giant multinucleated cells, similar to those found in the giant cell tumor at the bone level. Histologically, some authors consider that GCTST is a strictly benign tumor, consisting of welldefined multinucleated histiocytes admixed with eosinophils, lymphocytes and scattered spindleshaped cells, or hemosiderin deposits in its structure, and tumor cells do not have mitosis or atypia. Other authors consider that GCTST is a type of low-grade sarcoma; this entity was named “malignant fibrous histiocytoma, giant cell type” due to the histological similarity with malignant fibrous histiocytoma. The case of a female patient, suspected of giant cell tumor of the brachioradialis tendon sheath was presented. The MRI aspect of this tumor is not the typical one. The MRI examination consisted of a series of sequences, with T1 and T2 weighted images, fat suppression sequence, performed in all three planes, axial, sagittal, and coronal. Also, the examination was performed native, after the administration of intravenous contrast substance, when the 3D multiplanar sequences were performed. The final diagnosis was the post-operative anatomopathological examination, which confirmed that it was a giant cell tumor. We present this case for its less frequent localization - forearm, and the interest it might have in surgical treatment.


1986 ◽  
Vol 11 (1) ◽  
pp. 149-150
Author(s):  
C. B. MUTALE ◽  
P. S. PATIL ◽  
J. B. PATEL

A case of low grade malignant fibrous histiocytoma (MFH) arising from a metacarpal bone in a 21 year old African female is presented. The rarity of this disorder is emphasized with review of the relevant literature.


2010 ◽  
Vol 51 (8) ◽  
pp. 1129
Author(s):  
Che Ron Kim ◽  
Kyeong Hyun Kim ◽  
Ji Myung Yoo ◽  
Seong Wook Seo

2017 ◽  
Author(s):  
Ann Y Lee

Both myxofibrosarcoma and undifferentiated pleomorphic sarcoma (UPS) are sarcomas that primarily occur in the extremities of older patients. UPS (previously known as malignant fibrous histiocytoma) is now considered a diagnosis of exclusion, representing a group of high-grade, heterogeneous tumors with no clear line differentiation and characterized by high rates of distant metastases. Myxofibrosarcoma (formerly myxoid variant of malignant fibrous histiocytoma) is a distinct histologic entity that is largely distinguished from UPS by its myxoid stroma and curvilinear vascular pattern. Myxofibrosarcoma tends to have a locally infiltrative behavior that can often be seen as T2 hyperintense “tails” on magnetic resonance imaging. Local recurrence rates can be as high as 61% and are independent of grade. Unlike other sarcomas, it is recommended that tumors be excised with at least a 2 cm margin around both clinically and radiographically evident tumor. Low-grade tumors do not tend to metastasize but can recur as high-grade tumors with increased metastatic potential. Surgery is the mainstay of treatment for localized disease. For high-grade tumors, this may require complex plastics and vascular reconstruction. Selecting cases for multimodality therapy, including radiation and chemotherapy, should be done on a case-by-case basis after review by a multidisciplinary sarcoma team with particular attention to a patient’s risk of local recurrence and distant metastases. Further studies into histology-specific therapeutic targets will help inform future clinical trials. This review contains 4 figures, 4 tables and 54 references Key words: chemotherapy, distant metastases, extremity sarcoma, local recurrence, malignant fibrous histiocytoma, myxofibrosarcoma, radiation, sarcoma, surgical margins, T2 hyperintense tails, undifferentiated pleomorphic sarcoma


Rare Tumors ◽  
2016 ◽  
Vol 9 (1) ◽  
pp. 14-17 ◽  
Author(s):  
Shinichirou Yoshida ◽  
Takashi Murakami ◽  
Kentarou Suzuki ◽  
Shigemi Itou ◽  
Munenori Watanuki ◽  
...  

Adamantinoma is a rare, low-grade, malignant bone tumor. It frequently occurs in the tibia but rarely arise in the distal end of the fibula. This study reported a case of adamantinoma arising in the distal end of the fibula, resulting in good prognosis. A 38-year old female felt left ankle pain, and was suspected as having a bone tumor at the distal end of the fibula by X-ray. She was diagnosed as the classical adamantinoma of the fibula by open biopsy. En bloc wide resection of the tumor, primary arthrodesis of the ankle was performed. During the follow-up period of 7 years after the surgery, she has lived without any metastasis and local recurrence. A wide resection and arthrodesis of the ankle joint can provide a good outcome for adamantinoma arising in the end of the fibula.


2017 ◽  
Author(s):  
Ann Y Lee

Both myxofibrosarcoma and undifferentiated pleomorphic sarcoma (UPS) are sarcomas that primarily occur in the extremities of older patients. UPS (previously known as malignant fibrous histiocytoma) is now considered a diagnosis of exclusion, representing a group of high-grade, heterogeneous tumors with no clear line differentiation and characterized by high rates of distant metastases. Myxofibrosarcoma (formerly myxoid variant of malignant fibrous histiocytoma) is a distinct histologic entity that is largely distinguished from UPS by its myxoid stroma and curvilinear vascular pattern. Myxofibrosarcoma tends to have a locally infiltrative behavior that can often be seen as T2 hyperintense “tails” on magnetic resonance imaging. Local recurrence rates can be as high as 61% and are independent of grade. Unlike other sarcomas, it is recommended that tumors be excised with at least a 2 cm margin around both clinically and radiographically evident tumor. Low-grade tumors do not tend to metastasize but can recur as high-grade tumors with increased metastatic potential. Surgery is the mainstay of treatment for localized disease. For high-grade tumors, this may require complex plastics and vascular reconstruction. Selecting cases for multimodality therapy, including radiation and chemotherapy, should be done on a case-by-case basis after review by a multidisciplinary sarcoma team with particular attention to a patient’s risk of local recurrence and distant metastases. Further studies into histology-specific therapeutic targets will help inform future clinical trials. This review contains 4 figures, 4 tables and 54 references Key words: chemotherapy, distant metastases, extremity sarcoma, local recurrence, malignant fibrous histiocytoma, myxofibrosarcoma, radiation, sarcoma, surgical margins, T2 hyperintense tails, undifferentiated pleomorphic sarcoma


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