Giant intrapulmonary solitary fibrous tumor.

Solitary fibrous tumor (SFT) is a soft tissue tumor of mesenchymal origin involving, most commonly, the pleura. Intrapulmonary SFT is a slow-growing tumor that rarely reaches giant forms. SFTs are asymptomatic and often randomly discovered by routine chest X-rays. The diagnosis requires histopathological and immunohistochemical (IHC) examinations. Most of the SFTs are benign and present an indolent course. Larger tumors are more likely to be malignant and consequently associated with a worse prognosis. Despite having histopathological criteria for malignancy, the behavior of SFTs is challenging to predict. We report a case of giant intrapulmonary SFT of intermediate risk.

Resection of pleural solitary fibrous tumors with distinct feeding vessels arising from the descending aorta: a case report.

BACKGROUND: Pleural solitary fibrous tumors (pSFTs) are rare mesenchymal pleural tumors with rich vascularity. Surgical resection is the cornerstone of pSFTs treatment, requiring careful preoperative imaging to delineate lesion extent and vascular supply including contrast-enhanced computed tomography and other examinations depending on its size and characteristics. CASE PRESENTATION: The patient was a 34-year-old female with a mass measuring approximately 67 × 42 × 65 mm in the left posterior mediastinum. Intraoperatively, the mass demonstrated rich vascularity. Two veins originating from the abdominal cavity entered the lower pole, one converged from the superior pole, draining into the brachiocephalic vein. Additionally, two arteries arose directly from the descending aorta, while several veins drained into the intercostal veins. In response to unexpected intraoperative vascular findings, vascular clips and silk threads were used to ligate them. Subsequently, the tumor was successfully dissected, with approximately 600 ml of blood loss recorded during the 4-hour surgery. The patient exhibited a satisfactory postoperative recovery, and follow-up spanning over six months revealed no indications of recurrence or metastasis. CONCLUSIONS: We firstly present a case of successful resection of a pSFT in a 34-year-old woman with a distinct feeding vessel arising from the descending aorta and describe the related surgical procedures. This case highlights preoperative evaluation of mass vascularity based on contrast-enhanced computed tomography. When blood supply is challenging to clarify, angiography can offer additional details, especially for giant pSFTs. Despite this, thorough intraoperative exploration remains essential to detect unexpected vessels. Appropriate interventions should be customized based on the vascular origins and the surrounding anatomical structures.

A Rare Solitary Fibrous Tumor of the Mesorectum: A Case Report.

Solitary fibrous tumors (SFTs) are rare tumors that predominantly occur in the mesorectum. Few case reports have been published on mesorectal tumors, and this is the seventh case report. A 49-year-old female patient presented with a hypervascularized mesorectal tumor discovered incidentally during a routine medical examination. Using preoperative three-dimensional computed tomography (3D-CT), we identified vessels originating from the superior rectal and lateral sacral arteries, which are important sources of nutrients, and performed the procedure safely and without bleeding. Considering the lack of preoperative diagnosis and rectal blood flow, high anterior resection was performed. The histopathological diagnosis confirmed SFT, and the patient is currently doing well with no recurrence. Although SFT of the mesorectum occurs infrequently, it should be included in the differential diagnosis. In addition, the usefulness of preoperative 3D-CT, including the arterial phase, has been emphasized in such cases. This is the seventh reported case of a rare SFT in the mesorectum. Currently, there is no literature highlighting the usefulness of 3D-CT for SFTs of the mesorectum. However, it is a valuable preparatory tool for preoperative evaluation.

Exploring Solitary Fibrous Tumors at a Tertiary Cancer Center: Clinicopathological and Immunomorphologic Profile.

Background Solitary fibrous tumor (SFT) is a distinct fibroblastic tumor that can occur at any anatomical site and can manifest a variety of histopathological features. NAB2-STAT6 gene fusion has recently emerged as a sensitive and specific molecular marker and its surrogate on immunohistochemistry, STAT6 has also displayed considerable efficacy. Nevertheless, its histologic diversity can result in diagnostic challenges, especially when classic features are not apparent. Methods A retrospective study was conducted at a tertiary cancer centre in North India over 3 years to document the clinicopathologic and immunomorphologic profile of SFTs. Immunohistochemical analysis of BCOR and p53 were gauged additionally and patients were stratified according to Modified Demicco and Salas criteria for risk of metastasis. Results Sixteen patients of SFT were identified, affecting middle-aged men and women equally. Though lung/pleura are known to be involved commonly, SFT affects other sites such as the kidney, brain, buccal mucosa, liver, and penis as well. The majority endured localized disease while a lesser number suffered locoregional/distant spread. Two patients revealed features of a malignant profile. Risk stratification according to the Modified Demicco and Salas criteria evinced comparable results. No discernible relationship however was highlighted between the immunohistochemical expression of BCOR, p53, and any significant SFT parameter. Conclusion Although SFTs are very rare substantially benign mesenchymal neoplasms, pathologists must be conversant with their histological diversity and be vigilant of their malignant attributes. The worth of STAT6 immunohistochemistry for precise diagnosis and long-term studies for delineating clinical behavior cannot be overemphasized.

Endobronchial solitary fibrous tumors: An enigma for diagnosis.

Solitary fibrous tumors (SFTs) are rare mesenchymal neoplasms constituting less than 2% of all soft tissue tumors. They typically originate in the thoracic cavity, mainly in the pleura, but can also occur in other various sites such as lung parenchyma, pericardium, and bronchus. In this study, a 49-year-old non-smoking female with a history of allergies presented to our pulmonary clinic with a chronic cough. An explorative bronchoscopy revealed an intrabronchial mass in the left superior bronchi, and a 68 Ga-DOTATOC positron emission computed tomography suggested a carcinoid tumor. Subsequent pulmonary segmentectomy unveiled a well-circumscribed polypoid lesion diagnosed as a low-grade bronchus SFT through histopathological and immunohistochemical assessments. The patient was asymptomatic after surgical excision and showed no other lesion during the 6-month follow-up. The endobronchial location of SFT is uncommon, with only a few reported cases in the literature, underscoring the necessity of considering various differential diagnoses, including carcinoid, mucoepidermoid carcinoma, endobronchial pleomorphic adenoma, hamartoma, leiomyoma, and metastasis, depending on location and imaging features. This report underscores the importance of careful histological and immunohistochemical evaluation in understanding and appropriately stratifying the risk associated with polypoid lesions.

Solitary fibrous tumor in the temporalis muscle: a case report and literature review.

Solitary fibrous tumor (SFT) is an infrequently occurring neoplasm most commonly observed in the pleura, but it can develop in the head and neck region in occasional cases. However, no reports have described SFT in the temporalis muscle. Herein, we present the first known case of SFT in the temporalis muscle. A 47-year-old man complained of a painless palpable mass on his right temple. Facial enhanced computed tomography identified a 4.0× 2.9× 1.4 cm mass presenting as a vascular tumor in the right temporalis muscle under the zygomatic arch. The mass was excised from the right temporalis muscle under general anesthesia. A histopathologic examination revealed that the mass was an SFT. No complications occurred after surgery, including functional disability or sensory loss. The patient was followed up for 3 months without complications. Although SFT in extrapulmonary regions is rare, it should be considered in the differential diagnosis of masses that occur in the temporal area.

Solitary Fibrous Tumor in the Retroperitoneal Space Arising from the Diaphragm.

BACKGROUND/AIM: We present a case of solitary fibrous tumor, arising from the diaphragm in the retroperitoneal space, that was resected with robotic assistance. CASE REPORT: An 85-year-old female patient was referred to our hospital for evaluation of a suspected right renal tumor. Abdominal contrast-enhanced computed tomography revealed a tumor (maximum diameter, 36 mm) protruding from the superior pole of the right kidney. The patient was scheduled for robot-assisted, retroperitoneoscopic, partial nephrectomy based on a preoperative diagnosis of renal cell carcinoma. Intraoperative findings revealed that the tumor originated from the diaphragm and had no continuity with the renal parenchyma. Pathological examination revealed a solitary fibrous tumor. CONCLUSION: Solitary fibrous tumors are rare soft-tissue neoplasms with a distinct molecular feature of the fusion of nerve growth factor-inducible A gene-binding protein 2 with signal transducer and activator of transcription 6 gene (NAB2::STAT6). We believe that this is the first reported case of a solitary fibrous tumor arising from the diaphragm in the retroperitoneal space.

Recurrence of solitary fibrous tumor of the pleura with hypoglycemia (Doege-Potter Syndrome): a case report description.

Hypoglycemia has multiple causes, but the most common is a complication of insulin treatment. In addition to insulin therapy, tumors such as insulinomas of pancreatic origin and extrapancreatic tumors causing paraneoplastic syndromes should also be considered. Solitary fibrous tumors of the pleura (SFTP) is rare tumor, which when associated with hypoglycemia causes Doege-Potter syndrome. This article reports a case of a 69-year-old man with Doege-Potter syndrome and underwent the first surgical resection for SFTP. However, the tumor recurred 9 years later with hypoglycemic symptoms and implant metastasis. This recurrent tumor originated from the visceral pleura, was more aggressive and invaded the diaphragm and parietal pleura. After the second surgical removal of the tumor, the hypoglycemic symptoms disappeared.

Tumor fibroso solitario cerebral: A propósito de un caso / Solitary fibrous tumor of the brain: A case report

RESUMEN Introducción: Los tumores fibrosos solitarios (TFS) son neoplasias raras de origen mesenquimal que, aunque generalmente se desarrollan en la pleura visceral, ocasionalmente se presentan en la cavidad intracraneal. Además, se caracterizan por altas tasas de metástasis y recurrencia. Caso clínico: Se presenta el caso de un paciente masculino de 59 años con cuadro de 3 meses de cefalea y bradipsiquia. La tomografía reveló una tumoración neoformativa que infiltra cavidad nasal, celdillas etmoidales, y fosa craneal anterior, comprometiendo lóbulo frontal izquierdo. El paciente fue sometido a dos craniectomías exploratorias donde se realizaron los diagnósticos sugestivos de neoplasia glial de alto grado y TFS. Para la precisión diagnóstica se realizó inmunohistoquímica que fue compatible con tumor fibroso solitario. Se analiza el caso centrándose particularmente en los aspectos histopatológicos, localización inusual de este tumor y sus manifestaciones clínicas variables.

ABSTRACT Introduction: Solitary fibrous tumors (SFTs) are rare mesenchymal neoplasms that, although typically develop in the visceral pleura, occasionally occur in the intracranial cavity. Furthermore, they are characterized by high rates of metastasis and recurrence. Case Report: We present the case of a 59-year-old male patient with a 3-month history of headache and bradyphrenia. Computed tomography revealed a neoformative tumor infiltrating the nasal cavity, ethmoid sinuses, and anterior cranial fossa, involving the left frontal lobe. The patient underwent two exploratory craniectomies, during which diagnoses suggestive of high-grade glial neoplasia and SFT were made. For precise diagnosis, immunohistochemistry was performed, which was consistent with solitary fibrous tumor. The case is analyzed, focusing particularly on histopathological aspects, the unusual location of this tumor, and its variable clinical manifestations.

Surgical removal of an unusual huge solitary fibrous tumor in the mediastinum: a case report.

BACKGROUND: Intrathoracic Solitary Fibrous Tumors (SFT) mainly arise from the pleura; however, these tumors may also originate from the mediastinum. We present a rare case of posterior SFT extending to several mediastinal sites and with an unusual large size, successfully treated with surgical resection. CASE PRESENTATION: A 66-year-old female presented with an initial manifestation of ambiguous pain in the chest and dysphagia and later developed pitting edema in both lower extremities and cachexia five months before admission. Chest imaging confirmed a mediastinal mass (17 × 15 × 8 cm) which was surgically removed. Immunohistochemistry confirmed the diagnosis of a solitary fibrous tumor with positive B-cell lymphoma 2, STAT6, and CD99, negative S100 and smooth muscle actin, and low levels of Ki67 (5-7%). The patient's follow-up course was unremarkable. CONCLUSION: Mediastinal SFTs may grow extremely huge, with the potential to invade multiple adjacent sites. Surgical removal of the tumor remains the mainstay of treatment in these cases.

Surgery for Solitary Fibrous Tumors of the Pleura: A Review of the Available Evidence.

Solitary fibrous tumors of the pleura (pSFT) are a relatively rare neoplasms that can arise from either visceral or parietal pleura and may have different aggressive biological behaviors. Surgery is well known to be the cornerstone of the treatment for pSFT. We reviewed the existing literature, focusing on the role of surgery in the management and treatment of pSFT. All English-written literature has been reviewed, focusing on those reporting on the perioperative management and postoperative outcomes. Surgery for pSFT is feasible and safe in all experiences reported in the literature, but surgical approaches and techniques may vary according to the tumor dimensions, localization, and surgeons' skills. Long-term outcomes are good, with a 10-year overall survival rate of more than 70% in most of the reported experiences; on the other hand, recurrence may happen in up to 17% of cases, which occurs mainly in the first two years after surgery, but case reports suggest the need for a longer follow-up to assess the risk of late recurrence. Malignant histology and dimensions are the most recognized risk factors for recurrence. Recurrence might be operated on in select patients. Surgery is the treatment of choice in pSFT, but a radical resection and a careful postoperative follow-up should be carried out.

Surgical treatment for extremely rare solitary fibrous tumors of the central nervous system originating from cranial nerve VIII: new clinicopathological findings. Illustrative case.

BACKGROUND: Reports of solitary fibrous tumors (SFTs) of the central nervous system (CNS) originating from cranial nerves are extremely rare. The origins of these neurogenic SFTs of the CNS have been determined only by intraoperative findings, and there is no pathological evidence of whether they really originated from cranial nerves. OBSERVATIONS: A 54-year-old female with hearing loss and facial paralysis presented with a giant right cerebellopontine angle tumor. She was diagnosed with a petrous meningioma based on preoperative imaging, and the tumor was removed via the retrosigmoid approach after embolization of the tumor-feeding vessels. Intraoperatively, the tumor was not attached to the dura mater but extended from the internal auditory canal to the cisternal portion. The acoustic nerve was not identified, but it was possible to separate the tumor from the facial nerve. The tumor was removed as an acoustic schwannoma intraoperatively. Postoperative pathological examination revealed an SFT. Immunostaining revealed peripheral nerve bundles entrapped within the tumor tissue. The patient was diagnosed with an SFT of the CNS originating from the acoustic nerve. LESSONS: A neurogenic SFT of the CNS was diagnosed based on both intraoperative and pathological findings.

Impact of extent of resection and postoperative radiotherapy on survival outcomes in intracranial solitary fibrous tumors: a systematic review and meta-analysis.

The knowledge of optimal treatments for patients with intracranial solitary fibrous tumor (SFT) is limited, with inconclusive results from previous studies. In this study, we conducted a meta-analysis of relevant studies to identify the prognostic impact of the extent of resection (EOR) and postoperative radiotherapy (PORT) on survival outcomes of patients with intracranial SFT. We searched the Medline, Embase, and Cochrane Central Register of Controlled Trials (CENTRAL) to identify relevant studies published till April 2022. Progression-free survival (PFS) and overall survival (OS) were the outcomes of interest. Differences between two cohorts (gross total resection [GTR] vs. subtotal resection [STR] and PORT vs. surgery only) were estimated by calculating hazard ratios. Twenty-seven studies were selected for the meta-analysis, including data of 1348 patients (GTR, n = 819 vs. STR, n = 381 and PORT, n = 723 vs. surgery only, n = 578). Pooled hazard ratios of PFS (1, 3, 5, and 10 years) and OS (3, 5, and 10 years) revealed that the GTR cohort showed sustained superiority over the STR cohort. In addition, the PORT cohort was superior to the surgery-only cohort with respect to all PFS periods. Although the 10-year OS between the two cohorts was not statistically different, PORT showed significantly better 3- and 5-year OS than surgery only. The study findings suggest that GTR and PORT provide significant benefits for PFS and OS. Aggressive surgical resection of tumors to achieve GTR followed by PORT should be implemented as optimal treatments for all patients with intracranial SFT when feasible.

CT of retroperitoneal solitary fibrous tumor.

Solitary fibrous tumors are rare tumors of pluripotent fibroblastic or myofibroblastic origin that generally arise among older individuals, with a mean age of onset ranging from 55 to 65 years. Though typically associated with pleural involvement, solitary fibrous tumors can emerge in virtually every anatomic location within the body. Although most solitary fibrous tumors are benign, approximately 20% may exhibit malignant features such as local invasion, recurrence, and metastases. In this article, we report the case of a 58-year-old male with a diagnosis of a retroperitoneal solitary fibrous tumor. We analyze computed tomography imaging findings and additionally correlate imaging features with the patient's unique pathological and genotypic findings to optimize diagnosis.

Solitary Fibrous Tumors of the Lung: A Clinicopathological Analysis of 52 Cases.

OBJECTIVE: To explore the clinicopathological features of solitary fibrous tumors (SFTs) of the lung. METHODS: We collected the clinical data of 52 patients with SFTs of the lung confirmed by pathology, and summarized the clinical, radiological, and morphological features, the immunophenotypes, and the prognosis of SFTs. RESULTS: Fifty-two cases of SFTs of the lung were enrolled in this study, including 51 cases of borderline and 1 case of malignancy, 22 males and 30 females. The average onset age was 52.7 years. The lower lobe of the left lung was the preferred site of SFTs, accounting for 30.77% (16/52). Chest CT showed regular and well-demarcated soft tissue density mass, and the tumor size of most cases (46/52, 88.46%) was 1-10 cm. Morphological features: The distribution of tumor cells showed sparse and dense areas. Tumor cells were spindle-shaped, in whorls or hemangiopericytoma-like conformation. Atypia, mitotic figures, and necrosis were found. Immunohistochemistry showed positive expression of CD34, STAT6, Vimentin, BCL2, and CD99. Ki-67 was ≤ 5% in borderline SFTs and >20% in a malignant SFT. CONCLUSIONS: Solitary fibrous tumors of the lung occur more frequently in middle-aged and elderly people, and there is no significant difference in gender. The lower lobe of the left lung is the preferred site of SFTs. The size of most SFTs is 1-10 cm. Chest CT shows morphologically regular and well-demarcated soft tissue density mass. Pathologically, SFTs of the lung are mostly borderline and occasionally malignant. Immunohistochemistry shows the positive expression of CD34, STAT6, Vimentin, BCL2, and CD99.

Solitary Fibrous Tumor of the Umbilical Region in a Pediatric Patient.

Background: Fibrous tumors are rare tumors of mesenchymal origin arising in the serosal surfaces within the body. Although commonly seen in adults, solitary fibrous tumors rarely occur in children. Histopathology and immunohistochemistry are the methods of choice for diagnosing solitary fibrous tumors. Case Report: A 2-year-old male presented with a swelling over the umbilicus for the prior 8 months. The umbilical mass was excised and sent for histopathologic examination. The skin-covered greyish soft tissue mass measured 6 × 5.5 × 4.5 cm, and the cut surface showed a homogenous greyish growth. On microscopic examination, a predominantly well-circumscribed encapsulated tumor was noted, with spindle shaped cells arranged in a haphazard manner and ectatic vascular channels. The cells were immunoreactive for CD34 and signal transducer and activator of transcription 6 (STAT6) and negative for smooth muscle actin, desmin, myogenin, MyoD1, CD99, epithelial membrane antigen, and beta-catenin. Conclusion: The aim of this case is to make clinicians aware of the umbilicus as a rare site of solitary fibrous tumor in children and the diagnostic importance of STAT6.

Doege-Potter syndrome associated to metastatic solitary fibrous tumor.

Solitary fibrous tumor (SFT) is a rare fibroblastic mesenchymal neoplasm with an estimated annual incidence of 0.35 per 100,000 individuals. Doege-Potter syndrome is a paraneoplastic syndrome related to solitary fibrous tumor clinically characterized by hypoglycemia, occurring in less than 5% of cases. Herein, we report a case of metastatic SFT associated with recurrent severe hypoglycemia. A 43-year-old male with a noncontributory medical history presented with a painless and progressive growing mass in the right thigh. The histological evaluation rendered the diagnosis of SFT, and tumor resection was performed. One year after the operation, on the oncological follow-up, he was admitted to the emergency unit, manifesting an early-morning seizure associated with a severe hypoglycemia. The laboratory findings of non-islet cell tumor hypoglycemia (NICTH) in the background of a relapsed metastatic solitary fibrous tumor were consistent with the diagnosis of Doege-Potter syndrome. Hepatic embolization associated with oral glucocorticoid was an efficient palliative treatment to control the hypoglycemic crisis and allow hospital discharge.

Solitary fibrous tumor of the tongue.

Solitary fibrous tumor (SFT) is a benign mesenchymal neoplasm originally described in pleura with a rare presentation in the oral cavity. Herein, we report a case of a 28-year-old male patient who presented an asymptomatic slow-growing mass in the anterior part of the tongue. Intraoral examination revealed a well-circumscribed mass covered by normal mucosa with a fibrous consistency. Due to non-specific clinical findings, the initial diagnostic hypotheses include benign submucosal neoplasms such as leiomyoma, neurofibroma, SFT, and others. An excisional biopsy was performed. Microscopically, the tumor was surrounded by a thick fibrous capsule; hypo and hypercellular areas were arranged in a storiform pattern with a stroma formed by collagen and abundant vascularization. Tumor cells showed immunopositivity for CD34 and STAT-6 and no expression of CD99, AML, S-100, and Ki-67. According to these findings, the diagnosis of SFT was established. After 24 months, the patient is asymptomatic and has no evidence of recurrence. Although oral involvement is rare, SFT should be included in the differential diagnosis of oral submucosal lesions.

A Rare Occurrence of an Extensive Sino-Nasal Solitary Fibrous Tumour.

Introduction: Solitary fibrous tumours are uncommon in head and neck region, especially in the nasal cavities and paranasal sinuses, with most cases reported in the thoracic region in the pleura. It is often considered a borderline or low-grade malignant soft tissue tumour. Complete surgical resection is currently the treatment of choice, though intracranial and orbital extension of these lesions must be carefully evaluated and navigated to ensure a safe outcome. Case Report: A 36 years-old lady presented with a long one-year history of left-sided nasal obstruction with facial pain, headaches and mild visual disturbances. She had been treated for sinusitis for a prolonged period. Clinically, there was a left nasal mass obliterating the ostiomeatal complexes and the roof of the nasal cavity. MRI showed heterogeneously enhancing mass occupying the left ethmoid sinuses extending laterally eroding the left lamina papyracea to the orbit, medially towards the right nasal cavity eroding the nasal septum, and superiorly to extend intracranially. After inconclusive biopsies were performed, the mass was excised with a combined endoscopic and open lateral rhinotomy approach with left medial maxillectomy and reconstruction of the skull base defect. The tumour was eventually reported as a solitary fibrous tumour. Conclusions: Solitary fibrous tumour is a rare differential of tumours in the sino-nasal region, diagnosed via histopathology. Although generally slow-growing, these lesions may extend the adjacent structures namely the orbit and skull base. Definitive treatment via surgical resection may be performed safely after careful radiological assessment and multidisciplinary consideration.