Malignant solitary fibrous tumor of the kidney with IGF2 secretion and without hypoglycemia.

BACKGROUND: Solitary fibrous tumor (SFT) is a rare fibroblastic mesenchymal tumor that mostly involves the pleura and infrequently involves extra-pleural sites. De novo SFT of the kidney is uncommon, and malignant SFT is extremely rare. CASE PRESENTATION: We report a case of a 51-year-old man with a large malignant SFT in the left kidney. Pathological examination confirmed the diagnosis of SFT based on typical morphology, nuclear STAT6 expression, and NAB2-STAT6 gene fusion. The malignant subtype was determined by a large tumor size (≥ 15 cm) and high mitotic counts (8/10 high-power fields). KRAS mutation was identified by DNA sequencing. Insulin-like growth factor 2 (IGF2) was diffusely and strongly expressed in tumor cells, however, hypoglycemia was not observed. Hyperglycemia and high adrenocorticotropic hormone (ACTH) concentration were observed one month after surgery. Hormone measurements revealed normal blood cortisol and aldosterone levels, and increased urinary free cortisol level. A pituitary microadenoma was identified using brain magnetic resonance imaging, which may be responsible for the promotion of hyperglycemia. CONCLUSIONS: We report a case of renal malignant SFT with a KRAS mutation, which was previously unreported in SFT and may be associated with its malignant behavior. Additionally, we emphasize that malignant SFT commonly causes severe hypoglycemia due to the production of IGF2. However, this effect may be masked by the presence of other lesions that promote hyperglycemia. Therefore, when encountering a malignant SFT with diffuse and strong IGF2 expression and without hypoglycemia, other lesions promoting hyperglycemia need to be ruled out.

Intracranial Solitary Fibrous Tumor: A Case of a 21-Year-Old Male With Olfactory Hallucination.

Meningeal solitary fibrous tumors (SFTs) are a rare central nervous system neoplastic process, resulting in frequent misdiagnosis as meningioma prior to pathologic analysis. Appropriate diagnosis is essential to lowering morbidity and mortality, as Grade II or III SFTs are aggressive neoplasms that possess metastatic potential. The existing data may suggest that intracranial SFTs primarily afflict those in their fourth through sixth decades of life. However, we present the case of a patient outside this demographic presenting with symptoms that we were unable to identify in any prior reports. A 21-year-old male in the United States Navy presented to the emergency department (ED) with a two-month history of progressive headaches, leading to nausea and emesis. The patient also endorsed a daily incidence of the same olfactory hallucination followed by several minutes of palpitations, flushing, and dizziness. His neurologic exam was unremarkable, but imaging in the ED revealed a large mass abutting the right medial sphenoid wing. The radiographic appearance of the mass with a dural tail led to a preoperative diagnosis of meningioma. However, pathologic analysis following gross total resection identified the mass as an SFT. A brief literature review complementary to this case underscored the high variability of intracranial SFT case presentations with a relative scarcity of epidemiologic data due to rarity. This review identified that it was common to initially diagnose SFTs as meningioma, similar to this particular case. This emphasizes the importance of an appropriate pathologic diagnosis. This case adds to the existing literature as anecdotal evidence of SFT occurring in a young patient and a unique symptom profile most notable for olfactory hallucination and dysautonomia as features of focal seizure.

Thoracic solitary fibrous tumors with small cell features: A clinicopathological and immunohistochemical analysis of 5 cases.

Five cases of thoracic solitary fibrous tumor (SFT) with small cell features are presented mimicking a neuroendocrine neoplasm. The patients were four men and one woman aged 43 to 74 years who presented with symptoms of chest pain, cough, dyspnea or hemoptysis. Two tumors were intrapulmonary neoplasms, while three were pleural-based. Grossly, the tumors ranged in size from 4 to 6 cm and were white and solid; in two tumors necrosis was apparent. Histologically, they were characterized by a cellular proliferation composed of small cells with round nuclei and inconspicuous nucleoli. The cellular proliferation in some areas had a subtle nested pattern, while in other areas the tumor showed extensive sclerosis and small vessel proliferation. Cellular pleomorphism was not marked and the mitotic activity varied from 1 to 5 mitotic figures per 10 high power fields. Microscopically, necrosis was observed in two cases and focally present in one. Immunohistochemical stains showed tumors cells universally negative for pancytokeratin; in the two pulmonary cases, focal staining for synaptophysin, CD56, and INSM1 was observed. The unexpected lack of expression of pancytokeratin led to additional analysis revealing positive staining with CD34 and STAT6 confirming a diagnosis of SFT. Clinical follow-up showed tumor recurrence in one patient while three patients remained alive and well after a period of 12 to 20 months. The current cases highlight an unusual variant of SFT that may be confused with other small cell tumor entities, such as neuroendocrine or neuroectodermal tumors, especially when originating in the thoracic cavity.

Unveiling the Rarity: A Case Report on Solitary Fibrous Tumor of the Thyroid Gland.

Solitary Fibrous Tumor (SFT) rarely manifests within the thyroid gland, an organ predominantly associated with epithelial carcinomas. This case report explores the clinical narrative of a 70-year-old patient presenting with a sizable SFT localized in the left lobe of the thyroid, posing diagnostic challenges uncommon in thyroid nodules. The report delves into the clinical history, radiological findings, pathological assessments, and therapeutic interventions, contributing to the limited literature on thyroidal SFTs. The patient's ultrasound revealed a substantial thyroid mass causing tracheal and vascular displacement, categorized as TIRADS 3. Fine needle aspiration indicated mesenchymal origin, prompting further investigation. Contrast-enhanced computed tomography depicted a well-defined lesion with varied enhancement, compressing surrounding structures. Histopathology confirmed a spindle cell proliferation, prompting immunohistochemistry revealing CD34, STAT6, and Bcl-2 positivity, aligning with SFT characteristics. The rarity of thyroidal SFTs poses diagnostic challenges, necessitating reliance on immunohistochemistry for accurate differentiation from other spindle cell neoplasms. Radiological investigations, including ultrasound and magnetic resonance imaging, contribute to preoperative planning. The case underscores the importance of meticulous pathological examination, emphasizing the utility of immunohistochemistry in confirming SFT diagnosis. The report enhances understanding among clinicians, pathologists, and researchers, guiding improved diagnostic accuracy and tailored treatment strategies for future occurrences of thyroidal SFTs.

Reconstruction of Midface Defects after Radical Tumor Resection - a Rare Case Report of Solitary Fibrous Tumour of the Hard Palate.

Introduction- Spindle cell neoplasm is a variant of squamous cell carcinoma. One of its subtypes is solitary fibrous tumor. Its occurrence in head and neck is very rare and rarer in hard palate. But if occurs, radical excision is the only choice as it has malignant potential but coverage of such large mid face defects imposes a challenge in front of a Plastic Surgeon as it demands both soft tissue coverage and skeletal support. Report of the case- A 33 year male presented to our department with swelling of left side face involving the anterior palate, maxilla, nose, and upper lip. With the help of the surgical oncology team, wide local excision of the neoplasm along with bilateral infrastructure maxillectomy, total rhinectomy, total upper lip resection and total hard palatectomy was done. This created large defect in the mid face which was covered with free anterolateral thigh flap. Biopsy was done which revealed the swelling as a solitary fibrous tumor of hard palate. All the margins were free of tumour. The flap settled well. Nostrils were secured with nasal stents. After 3 months, an expander was placed in forehead of the patient for future nasal reconstruction. After 3 months, nasal reconstruction was done using expanded forehead flap and costal cartilage. After 21 days flap detachment and insetting was done. White roll creation was also done. One more secondary procedure was done for flap thinning as patient had complain of nasal obstruction. After 6 months vascularised free fibula bone graft was introduced to reconstruct maxilla for future dental rehabilitation. The patient is in regular follow up and he is satisfied with the results. Discussion- Mid face defects involving perioral and nasomaxillar areas are very uncommon and require composite reconstruction. In such cases, microvascular free flap coverage is an irreplaceable option. Multiple stages might have to be done for further refinement. Conclusion- Reconstruction after oncological resection is always very demanding. With proper preoperative planning and skilled execution, the patient can be benefited functionally, aesthetically and psychosocially.

Intracranial Solitary Fibrous Tumor/Hemangiopericytoma: A Series of 14 Cases and Review of the Literature.

Solitary fibrous tumor (SFT) is a rare type of tumor characterized by spindle-shaped cells originating from mesenchymal tissue. This case series presents a collection of 14 intracranial solitary fibrous tumors treated between 2014 and 2022 in our institute in Bucharest, Romania. Through a systematic investigation, key aspects spanning the preoperative, intraoperative, and postoperative phases of patient care were highlighted. Our study examines various factors including tumor location (which was very heterogeneous), size (median of 49 mm, ranging between 22 mm and 70 mm), surgical techniques employed, and recurrence rates. The data was analyzed using Python version 3.10 (Python Software Foundation, Wilmington, Delaware, United States). Gender disparities in SFT were noted, particularly the male-to-female ratio which was 5:9. The use of the Medical Research Council (MRC) Scale for Muscle Strength aided in evaluating severity and postoperative outcomes. GTR was achieved in nine out of 14 cases (64.28%), prolonging the period of recurrence-free survival.

A rare case of solitary fibrous tumor of the lung parenchyma: case report.

Solitary fibrous tumor (SFT) of the lung is a rare neoplasm, usually originating from lung pleura. We present a case report of a 57-year-old male with no significant medical history who was incidentally diagnosed with an SFT of lung parenchyma on chest computed tomography scan. Radiological imaging revealed a well-defined mass in the left lower lobe of the lung. Biopsy and histopathological examination confirmed the diagnosis of solitary fibrous tumor. This case highlights the importance of considering SFT in the differential diagnosis of lung masses, as its clinical presentation and radiological features can mimic those of more common pulmonary malignancies.

Solitary fibrous tumor of the adrenal gland: a case report and review of the literature.

Solitary fibrous tumor (SFT) is a rare mesenchymal tumor, probably of fibroblastic origin, mainly in the extremities and pleura. Primary SFT of the adrenal gland is clinically more rare. Here, we report the case of a 47-year-old woman who detected a left adrenal mass on physical examination, without any symptoms, and no laboratory abnormalities. A computed tomography (CT) examination of the adrenal gland suggested a round-like soft tissue density shadow in the left adrenal area. An unenhanced scan showed uneven density of the mass, with a scattered circular-like cystic low-density shadow inside, and an enhanced scan showed obvious uneven enhancement. We considered it to be adrenal pheochromocytoma. Ultimately, the patient was treated with laparoscopic left adrenalectomy. A pathological examination suggested an adrenal SFT. We reviewed previous case reports of adrenal SFTs and summarized the clinical characteristics of adrenal SFT combined with the relevant literature. For adrenal tumors with uneven low-density shadow and uneven CT enhancement features, we should consider the differential diagnosis of adrenal SFT.

Mesenchymal neoplasms of the tongue: A clinicopathologic study of 93 cases.

Neoplasms of the tongue are relatively common, and the vast majority are epithelial in phenotype. Although uncommon, a diverse and distinctive array of mesenchymal neoplasms arises in this anatomic site. To increase our understanding of these lesions, we reviewed our experience of MNs of the tongue and described their clinicopathologic features. The pathology archives from 2005 to 2021 and the consultation files of one of the authors were queried for all MNs of the tongue. We reviewed the histologic slides and ancillary studies and obtained clinical data from the available medical records. Ninety-three cases were identified, and they form the study cohort - to our knowledge, this is the largest series of mesenchymal neoplasms of the tongue. Forty-eight patients were female, and forty-five were male, with a mean age of 51 years (range: 1-94 years). The tumors included 43 (46.2%) hemangiomas, 14 (15%) granular cell tumors, 8 (9%) lipomas, 4 (4.3%) schwannomas, 4 (4.3%) solitary fibrous tumors - all with low risk of progression based on risk stratification criteria, 2 (2.2%) lymphangiomas, 3 (3.2%) Kaposi sarcomas, 2 (2.2%) chondromas, 2 (2.2%) myofibromas, 1 (1.1%) solitary circumscribed neuroma, 1 (1.1%) perineurioma, 1 (1.1%) neurofibroma, 1 (1.1%) ectomesenchymal chondromyxoid tumor, 1 (1.1%) atypical glomus tumor with a NOTCH2 rearrangement and TLL2 mutation, 1 (1.1%) spindle cell rhabdomyosarcoma, 1 (1.1%) pleomorphic fibroblastic sarcoma, 1 (1.1%) malignant rhabdoid tumor, 1 (1.1%) leiomyosarcoma, 1 (1.1%) angiosarcoma, and 1 (1.1%) alveolar soft part sarcoma. Most of the patients underwent surgical excision, and 1 patient (with hemangioma) underwent embolization. On follow-up, the patient with spindle cell rhabdomyosarcoma developed postoperative numbness at the surgical site and was disease-free through 17 months of follow-up. The patient with leiomyosarcoma declined adjuvant radiation and developed metastasis to the lung at 22 months. The patient with alveolar soft part sarcoma had metastases to the lung at the time of diagnosis and received adjuvant chemotherapy. The remaining patients had no local or distant recurrence. MNs of the tongue are usually benign and characterized by either endothelial, adipocytic, or schwannian differentiation. The mainstay of treatment is surgical excision with the extent of excision determined by tumor type. Adjuvant therapy is reserved for high-grade sarcomas.

Solitary fibrous tumor within the mesorectum: literature review based on a case report of resection by transanal minimally invasive surgery (TAMIS).

PURPOSE: Solitary fibrous tumors (SFT) are a rare entity of in majority benign neoplasms. Nevertheless, up to 20% of cases show a malignant tendency with local infiltration or metastasis. Commonly arising in the thoracic cavity, only few cases of SFT of the mesorectal tissue have been reported in the literature. Complete surgical resection, classically by posterior approach, is the treatment of choice. The purpose of this review is to demonstrate the safety and suitability of transanal minimally invasive surgery (TAMIS) as a surgical approach for the resection of benign pararectal solid tumors. METHODS: We report the case of a 52-year-old man who was diagnosed incidentally with SFT of the distal mesorectum. Resection by TAMIS was performed. Based on this case, we describe the steps and potential benefits of this procedure and provide a comprehensive review of the literature. RESULTS: Histopathology confirms the completely resected SFT. After uneventful postoperative course and discharge on day four, follow-up was recommended by a multidisciplinary board by clinical examination and MRI, which showed a well-healed scar and no recurrence up to 3 years after resection. CONCLUSION: SFT of the mesorectum is a very rare entity. To our knowledge, this is the first report on a TAMIS resection for SFT, demonstrated as a safe approach for complete resection of benign pararectal solid tumors.

[Clinical analysis of 12 cases of solitary fibrous tumors in nasal cavity, sinuses and skull base].

Objective:To summarize and analyze the clinical manifestations, diagnosis and management and prognostic features of solitary fibrous tumor（SFT） in nasal cavity, sinus and skull base. Methods:The clinical data of 12 patients with STF from nasal cavity, sinus and cranial base admitted to the Affiliated Hospital of Qingdao University from April 2014 to January 2022 were retrospectively analyzed, including 4 patients admitted to the department of Otolaryngology head and neck surgery and 8 patients admitted to the department of skull base surgery The clinical characteristics, diagnosis, management and prognosis were analyzed. Results:Twelve patients were included in this research, including 7 males and 5 females. All patients received surgical treatment, and 4 patients also received postoperative adjuvant chemoradiotherapy. After follow-up for 12-60 months, 4 patients with adjuvant radiotherapy and chemotherapy had a good prognosis, and among 8 patients who did not receive radiotherapy and chemotherapy, 6 patients had good prognosis and 2 patients showed relapse. Four patients with a history of recurrence of SFT after surgery were admitted to our hospital for surgical treatment, in which 1 patient had relapse after surgery, and none had metastasis. Nasal cavity and sinus to skull base SFT is rare. The most effective treatment for this disease is surgical resection, and postoperative adjuvant chemoradiation and long-term follow-up can achieve a better prognosis. En bloc resection is the key to treatment success.

Expression of fibroblast activation protein in intrathoracic solitary fibrous tumor.

PURPOSE: Fibroblast activation protein (FAP) is a serine integral membrane protease, the expression of which has been confirmed in various cancer types. Solitary fibrous tumors of the pleura (SFTP) are rare mesenchymal fibroblastic neoplasms. We present a case of 18F-labeled FAP inhibitor ([18F]FAPI-74) PET imaging and its correlation with histological FAP expression and review an SFTP series at our institution in relation to the extent of FAP expression. METHODS: This retrospective study included 13 patients who underwent surgery between March 2011 and December 2022 at our institute. One of the patients also underwent [18F]FAPI-74 PET imaging. We semi-quantitatively evaluated FAP expression in SFTPs using immunohistochemical staining and H-scores. RESULTS: Nine of the 13 patients were male, with a median age of 64 years (range, 28-79 years). The median tumor size was 6.6 cm (1.1, 16 cm). In the pathological findings, expression levels of Ki67 were 1-5% in 12 of 13 cases. Furthermore, FAP expression was observed in all patients, and the median H-score was 160 (range, 10-280). The H-score of FAP expression in two of the 13 patients was low (10 in both), and that in two of the 13 patients was high (240 and 280). The SUVmax value of [18F]FAPI-74 PET was 3.57 in a patient in whom the H-score of FAP expression was 180. CONCLUSIONS: SFTPs expressed FAP to varying degrees in different patients and the [18F]FAPI-74 PET results in one patient reflected FAP expression in the tumor tissue.

Unsuccessful laparoscopic resection of a large pelvic solitary fibrous tumor: A case report.

We present a rare case of a female pelvic solitary fibrous tumor unsuccessfully resected using single-port laparoscopy, requiring conversion to laparotomy. Although the resection was successful, the surgical approach could have been improved. For large tumors, minimally invasive results are possible with flexible choices of equipment and incision position.

Cardiac solitary fibrous tumor - an extremely rare but potentially fatal diagnosis.

The background of this review is a description of the case of a 28-year-old man with an extremely rare cardiac solitary fibrous tumor (SFT). Although this tumor was removed surgically and in the 6-month follow-up examination no relapse was noted, recurrence was observed and confirmed in the magnetic resonance imaging 4 months later. SFT prevalence, symptoms and signs, treatment options and prognosis are reviewed.

The Third-Time Recurrence of a Thoracic Solitary Fibrous Tumor in a Patient Presenting With Paraneoplastic Hypoinsulinemic Hypoglycemia (Doege-Potter Syndrome).

We present a case of a 76-year-old Caucasian female with a recurrent solitary fibrous tumor (SFT) of the pleura, showcasing a rare manifestation of hypoglycemia associated with Doege-Potter syndrome (DPS). Having undergone two previous surgeries for SFT, the patient presented to the emergency department with severe fatigue, recurrent episodes of loss of consciousness, and hypoglycemia, despite lacking a history of diabetes mellitus. Radiological evaluation revealed a substantial recurrent SFT in the left lung, prompting excision through a left posterolateral thoracotomy. Remarkably, the patient's altered mental status and hypoglycemia resolved postoperatively. The case meets the criteria for aggressive SFT behavior, warranting consideration for adjuvant radiation therapy to control the risk of recurrence. This report highlights the nuanced interplay between SFT recurrence, paraneoplastic syndromes like DPS, and the potential benefits of adjuvant therapeutic strategies in managing these complex clinical scenarios.

Solitary Fibrous Tumor With an Acute Subdural Hematoma: A Case Report and Review of the Literature.

Solitary fibrous tumor (SFT) is a rare interstitial tumor that originates from various soft tissues, and SFTs occurring within the cranium are extremely rare. While intracranial SFTs with cerebral hemorrhage or subarachnoid hemorrhage have been reported, there have been no reports of intracranial SFTs causing subdural hematoma. In this case, we report on an intracranial SFT accompanied by a subdural hematoma. A 29-year-old female was emergently transported due to the sudden onset of persistent headache and vomiting that began the night before. CT and MRI imaging revealed a hemorrhagic tumor under the tentorium and an acute subdural hematoma extending along the tentorium. The excised tumor was diagnosed as an SFT through histopathological examination. After undergoing radiation therapy, no recurrence has been observed. This is the first case report of an SFT accompanied by a subdural hematoma, and it is vital to recognize that SFTs can be associated with subdural hematomas for proper diagnosis and treatment planning.

Solitary Fibrous Tumor of Head and Neck Region: A Series of Three Cases at an Uncommon Location With a Review of the Literature.

Solitary fibrous tumors (SFTs) uncommonly involve the head and neck region. Head and neck SFTs (HNSFTs) exhibit diverse histological features and can mimic several neoplasms with different treatment and behavior. Herein, we report the clinicopathological features of three cases of HNSFT. Case 1 was a 29-year-old female who presented with a nasal cavity mass measuring 3.5 cm. The patient underwent surgical excision. Microscopic examination revealed classic histological and immunohistochemical (IHC) features of SFT. Unusual histological features included epithelioid morphology, clear cells, and edematous change. She developed local recurrence after 11 months, which was also treated with surgery. Case 2 was a 55-year-old male who developed a 1-cm mass at the buccal mucosa. Surgical excision of the tumor was performed. The tumor was completely circumscribed microscopically. Characteristic histological and IHC features of SFT were identified. Unusual histological features observed were an adenomatous pattern, clear cells, and myxoid change. The patient was alive and disease-free at the 12-month follow-up. Case 3 was a 59-year-old female presenting with a medial canthus mass measuring 1.4 cm. The patient underwent surgical excision. Histological and IHC features observed were diagnostic for SFT. Unusual histological features identified were wavy nuclei and multinucleated stromal giant cells. The patient was alive and disease-free at the 124-month follow-up. Diagnosis of SFT can be challenging in unusual locations like the head and neck region. In addition, the histological spectrum of HNSFT is diverse. Therefore, knowledge about unusual histological features and classic IHC expression is essential for establishing correct diagnosis. Long-term follow-up is recommended because of the risk of recurrence in HNSFT.

Parasagittal meningeal hemangiopericytoma/solitary fibrous tumor: Two case reports and a literature review.

Background: Solitary fibrous tumor/meningeal hemangiopericytoma (SFT/M-HPC) is a rare neoplasm which accounts for around 1% of the intracranial masses. This pathology has a high risk for recurrence and metastasis to distant locations such as the liver, lungs, and bones. Precise diagnosis necessitates detailed histopathological examination. Case Description: We present two case reports of SFT/M-HPC. The first case is a 44-year-old female who presented with headache, nausea, vomiting, and frontal ataxia for several months. Imaging findings showed a large parasagittal extra-axial mass with compression of the frontal horns of both lateral ventricles. She underwent gross total resection with an uncomplicated postoperative period. The patient had no recurrent tumors or distal metastases in the follow-up period of 5 years. The second case is a 48-year-old male who presented with right-sided hemianopsia and hemiparesis. Computed tomography (CT) scans revealed a large parieto-occipital extra-axial mass with superior sagittal sinus engulfment and dislocation of the interhemispheric fissure. He underwent gross total resection with an uncomplicated postoperative period. Six years later, he presented with right-sided weakness. CT scan showed a multifocal recurrent mass at the previous location. He underwent subtotal resection with an uncomplicated postoperative period. Conclusion: SFT/M-HPC should be considered when presented with a meningioma-like tumor mass on preoperative imaging. Immunohistochemical study is crucial for the correct diagnosis. Strict long-term follow-up examinations and regular magnetic resonance imaging scans are key to preventing the appearance of metastases and large recurrent masses.