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Sequential MRI imaging of hepatic hemangioma. Note delayed centripetal filling: (A) T1 arterial phase, (B) T1 60-s delay phase, (C) T1 postcontrast phase and (D) T1 hepatographic phase.
Source publication
Hepatic solitary fibrous tumor (HSFT) is a very rare benign liver tumor without well-defined findings on imaging. Even with multiphase advanced contrast-enhanced liver imaging, a definitive preoperative diagnosis is impossible. The diagnostic process can be further complicated when there are two concurrent lesions with different radiologic appearan...
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Citations
... It also might present with sign and symptoms of mass effect on adjacent visceral or neurovascular structure. There are reports of incidental findings in abdominal imaging studies (8,9). ...
... They were abdominal pain, vomiting, anorexia, abdominal distension, postprandial fullness, weight loss, and fatigue [10]. Hypoglycemia may be a finding, being part of the paraneoplastic syndrome in which there is production of insulin-like growth factor, tending to return to normal parameter after surgical treatment [11,12]. Biochemical laboratory tests do not tend to present variation [10]. ...
... The imaging tests are no diagnostic and do not determine the behavior of the neoplasm, as in most tumors with mesenchymal line, being helpful to exclude invasion and elucidate the relationship between the tumor and neighboring structures [13]. The admixed cellular and collagenous components likely to be responsible for the heterogeneous enhancement on imaging [11]. Abdominal ultrasound mostly reveals a well-defined, hypoechogenic, or hyperechogenic mass. ...
... The main concern in such cases is that SFT can develop malignant transformation. The risk of malignancy is not extremely low (around 10% of reported cases), meaning that SFTL carries a real risk of malignant transformation [10,11,14]. Univariate analysis indicates that nuclear atypia/pleomorphism, high mitotic index, extrapleural location, p53-positive staining, and the presence of telomerase reverse transcriptase (TERT) mutation are associated with lower survival rates [12]. ...
... The clinical presentation of SFTL is generally nonspecific, ranging from weight loss and fatigue to upper abdominal fullness [16] or discomfort due to the tendency of these tumours to be quite large. In many cases, SFTLs are found incidentally during routine examination [17][18][19][20][21][22] or on routine imaging while investigating other pathologies [2,[23][24][25]. Patients may also present with symptoms secondary to compression of visceral or neurovascular structures adjacent to the mass such as [26], postprandial pain/nausea/vomiting [22,[27][28][29] or jaundice [30]. ...
Background
Solitary fibrous tumours (SFT) are neoplasms of mesenchymal origin that predominantly arise from the pleura. SFT of the liver (SFTL) are a rare occurrence with little number of cases reported in English literature. Malignant cases of hepatic SFT are an even rarer occurrence. For this reason, the prognostic evaluation of SFTLs is unknown and difficult to measure. MethodsA search on English literature on “Solitary Fibrous Tumour of the Liver” was conducted on common search engines (PubMed, Google). All published articles, case reports and literature reviews and their reference lists were reviewed. Case reportThis paper presents a 61-year-old male who was referred to a tertiary hospital in April 2010 with marked hepatomegaly. USS, CT and MRI scans were suggestive of a neoplasm, and the patient underwent a subsegmental IVb resection in June 2010. The specimen demonstrated histological and immunohistochemical features of malignant SFTL with clear resection margins. The patient was followed up regularly for 3 years with imaging and no suggestion of recurrence. Six years after the initial surgery, the patient represented with worsening right upper quadrant pain and dyspnoea secondary to extensive tumour recurrence adjacent to the resection site and metastatic deposits in the pleura. The patient was managed symptomatically and discharged for community follow-up after palliative involvement. ConclusionsSFTL are rare with only 84 cases reported in the English Literature including the present case. The average age of patients is 57.1 and occurs in females more than males (1.4:1). Most SFTLs follow a benign course, however, 17.9% of cases displayed malignant histological features. Only three cases including the current case are reported to have both local recurrence and metastasis. Surgical resection remains the mainstay of treatment and appears to be curative of most cases. The rarity of this tumour makes it difficult to evaluate its prognosis and natural course.
Simple Summary
Extrameningeal solitary fibrous tumors (SFTs) are distinct mesenchymal neoplasms with the propensity for recurrence and the characteristic genetic marker of the NAB2-STAT6 fusion gene. These tumors typically present as slow-growing masses, most commonly in the extremities or trunk. Different clinical behaviors ranging from low to high aggressiveness with the potential for metastasis can be observed. Diagnosis is based mainly on immunohistochemical staging for classic CD34 and STAT6 markers, although it can be perplexed by the similarity to other lesions. Surgical resection remains the primary treatment, and long-term follow-up is obligatory due to the unpredictable nature of SFTs. Further research is emerging to understand the biological behavior and optimal management with efficient treatment of SFTs.
Abstract
Solitary fibrous tumors (SFT) are rare mesenchymal neoplasms that account for less than 2% of all soft tissue masses. In the latest WHO 2020 Classification of Soft Tissue Tumors, extrameningeal SFT was listed as intermediate (rarely metastasizing) or malignant neoplasms. Due to the lack of characteristic clinical features, their diagnosis and treatment remain challenging. The pathogenesis of SFT is often associated with the presence of fusions of the NAB2-STAT6 gene on the 12q13 chromosome. Cytoplasmic CD34 positive staining is considerably characteristic for most SFTs; less frequently, factor XII, vimentin, bcl-2, and CD99 are present. A key factor in the diagnosis is the prevalent nuclear location of STAT6 expression. Radical resection is the mainstay of localized SFTs. In the case of unresectable disease, only radiotherapy or radio-chemotherapy may significantly ensure long-term local control of primary and metastatic lesions. To date, no practical guidelines have been published for the treatment of advanced or metastatic disease. Classical anthracycline-based chemotherapy is applicable. The latest studies suggest that antiangiogenic therapies should be considered after first-line treatment. Other drugs, such as imatinib, figitumumab, axitinib, and eribulin, are also being tested. Definitive radiotherapy appears to be a promising therapeutic modality. Since standards for the treatment of advanced and metastatic diseases are not available, further investigation of novel agents is necessary.
A solitary fibrous tumor (SFT) is a rare mesenchymal tumor. Ex situ hepatectomy and liver autotransplantation are novel methods for the treatment of complicated liver tumors, for example, those involving vascular structures, including the inferior vena cava, which are unresectable by conventional approaches. The present study describes a rare case of a massive hepatic SFT in a 32-year-old female who underwent ex situ hepatectomy and liver autotransplantation to achieve a radical resection. The surgery was without complications. Post-operative histopathological and immunohistochemical examinations revealed an SFT of the liver. The patient was discharged 29 days after the surgery with fully recovered liver function. The routine check-up 3 months after surgery indicated normal liver function and no evidence of recurrence. Additionally, an exhaustive review of available literature was performed to provide a complete overview of the current status of SFTs. In summary, the present study found that ex situ hepatectomy and liver autotransplantation are suitable surgical techniques for treating a giant SFT, as well as other liver neoplasms that are considered unresectable by conventional surgery.
