Malignant Solitary Fibrous Tumor of the Humerus: A Case Report of an Extremely Rare Primary Bone Tumor

Suarez-Zamora, David A.; Rodríguez‐Urrego, Paula A.; Soto-Montoya, Camilo; Rivero-Rapalino, Oscar; Palau-Lazaro, Mauricio A.

doi:10.1177/1066896918780348

Cited by 9 publications

(7 citation statements)

References 14 publications

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“…The histological and molecular diagnostic criteria used in soft tissue SFT (S-SFT) have been recently applied on “non otherwise classified” primary bone tumors, drawing out a new category of SFT of the bone (B-SFT) [ 4 , 5 , 6 , 7 ]. Nevertheless, B-SFT is exceedingly rare, with only few cases are described in the literature [ 8 , 9 , 10 , 11 ], and its biological behavior has not yet been assessed. From a histopathological and molecular point of view, primary B-SFT shares the same features of S-SFT.…”

Section: Introductionmentioning

confidence: 99%

Clinical, Histological, and Molecular Features of Solitary Fibrous Tumor of Bone: A Single Institution Retrospective Review

Bianchi

Lana

Gambarotti

et al. 2021

Cancers

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Primary solitary fibrous tumor (SFT) of the bone is extremely rare, with only few cases reported in the literature. We retrieved all cases of primary SFT of the bone treated at our institution and we assessed the morphology and the immunohistochemical and molecular features to investigate the clinical outcome of primary SFT of the bone and any clinical relevance of clinical and histological criteria of aggressiveness currently adopted for the soft tissues counterpart. Morphologically, 15 cases evidenced high cellularity, cytologic atypia, and foci of necrosis and were associated with more than 4 mitotic figures/10 HPF. Immunohistochemical analysis showed an expression of CD34 and of STAT6 immunopositivity in 95% and in 100% of cases, respectively. The presence of NAB2-STAT6 chimeric transcripts was found in 10 out of 12 cases in which RT-PCR analysis was feasible, whereas TERT promoter mutations analysis was feasible in 16 cases and only a C-to-T substitution in a heterozygous state was found in one DNA sample for the C228T genetic variant. P53 variants were assessed in 12 cases: 11 (91.6%) cases showed a variation, while in one case, no alteration was found. Disease-specific survival was 64% at 5 years and 49% at 10 years. Statistical analysis showed no correlation between survival and all the clinicopathological and molecular parameters evaluated. In conclusion, at difference to SFT of soft tissues, aggressive behavior of primary SFT of the bone seems to be independent from mitotic count or any other clinicopathological and molecular features.

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Section: Introductionmentioning

confidence: 99%

Clinical, Histological, and Molecular Features of Solitary Fibrous Tumor of Bone: A Single Institution Retrospective Review

Bianchi

Lana

Gambarotti

et al. 2021

Cancers

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“…Unlike for SFT in the soft tissue, no dedifferentiated SFT are described in bone and there is no consensus on the definition of malignant SFT. Case reports of humerus [57,58] and spine [59] SFT, showed occurrence of long-term relapses after surgery [57,58]. These data indicate the need for long-term follow-up (>5 years) in case of bone SFT diagnosis and metastasectomy is recommended whenever possible.…”

Section: Spindle Cell Sarcomasmentioning

confidence: 84%

Rare Primary Malignant Bone Sarcomas

Palmerini

Righi

Staals

2020

Cancers

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Rare primary malignant bone sarcomas (RPMBS), other than osteosarcoma, chondrosarcoma, chordoma, and Ewing sarcoma, account for about 5–10% of primary bone tumors and represent a major diagnostic challenge. These tumors include spindle cell and round cell sarcoma entities, hemangiopericytoma-like and vascular tumors. Additionally, several histotypes, traditionally described in the soft tissues, such as myxofibrosarcoma, synovial sarcoma, and malignant peripheral nerve sheath tumor of bone, have been reported in patients with primary bone tumors. While wide surgical resection is the mainstay of local treatment, systemic therapy of these rare entities is controversial. Patients with undifferentiated spindle cell or pleomorphic high-grade tumors of bone, are usually treated with osteosarcoma-like chemotherapy, while patients with round cell and undifferentiated round cell tumors (URCTs), may respond to sarcoma treatment regimens for Ewing sarcoma patients. Studies on analogies and differences among these ultra-rare tumors have seldom been reported. This review describes relevance, clinical aspects, diagnostic procedures, staging, treatment recommendations, and current research in this composite tumor group.

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“…SFTs are rare mesenchymal neoplasms of fibroblastic origin that account for less than 2% of all soft tissue tumors and have an incidence of 0.2/100,000 people-year 7 . It typically has a peak age incidence in the adult life, with the mean age presentation during the 50-60 years and affects both sexes equally 7 . Although formerly believed to be restricted to the pleura, these tumors can be found all over the body 3 .…”

Section: Discussionmentioning

confidence: 99%

“…Klemperer et al 1 first described the SFT in the pleura in 1991. From 1991 until now, only five cases of intraosseous SFT have been reported, two involved the metaphysis of the humerus 7-8 , one the vertebral arch of the L1 vertebra 5 , one the occipital skull 4 and one the right ileum 6 . All patients arrived at the hospital referring local pain or with pathological fractures and the imaging studied revealed an osteolytic lesion without any specific diagnostic features.…”

Section: Discussionmentioning

confidence: 99%

Primary intraosseous solitary fibrous tumor: an extremely rare case report and brief review of the literature

Coppola¹,

Zoccali²,

Baldi³

et al. 2022

Pathologica

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Summary Solitary fibrous tumor (SFT), a rare mesenchymal neoplasm of fibroblastic origin, was initially discovered in the mediastinal pleura and then described in many extra-pleural sites. The reports of primary solitary fibrous tumor of bone are extremely rare and only a few cases have been previously mentioned in the literature, most of which in flat and short bones. Here we present the case of a 53-year-old female, who was referred to the emergency department of a peripheral hospital after an accidental fall. Imaging studies revealed an intertrochanteric fracture with an underlying intramedullary lytic lesion. A biopsy was performed and a diagnosis of Ewing sarcoma was initially suggested. She arrived at our hospital where we reevaluated the case. The biopsy was reviewed and a diagnosis of intraosseous SFT was proposed. She underwent en-block resection of the proximal right femur. Primary SFTs of the bone are, like in our case, easily misdiagnosed due to the low specificity of the imaging studies and the extreme rarity of the localization. An accurate diagnosis and early resection are very important and with careful long-term follow-up is essential, particularly in those who with malignant behavior, for the early detection of possible recurrence or metastasis.

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Malignant Solitary Fibrous Tumor of the Humerus: A Case Report of an Extremely Rare Primary Bone Tumor

Cited by 9 publications

References 14 publications

Clinical, Histological, and Molecular Features of Solitary Fibrous Tumor of Bone: A Single Institution Retrospective Review

Clinical, Histological, and Molecular Features of Solitary Fibrous Tumor of Bone: A Single Institution Retrospective Review

Rare Primary Malignant Bone Sarcomas

Primary intraosseous solitary fibrous tumor: an extremely rare case report and brief review of the literature

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