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Solitary fibrous tumors (SFTs) are rare mesenchymal neoplasms that can occur intraventricularly, presenting diagnostic and management challenges. We describe a case of a 21-year-old male with no significant medical history who presented with intermittent headaches and vomiting, progressing to continuous symptoms. Neurological examination was unremarkable. Brain MRI revealed an isointense lesion in the occipital horn of the left lateral ventricle, diagnosed as an SFT. Surgical excision via a transcortical approach was successful, followed by postoperative radiotherapy. This case highlights the complexities in diagnosing and treating intraventricular SFTs, emphasizing the need for comprehensive evaluation and multimodal management strategies.
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Solitary fibrous tumor (SFT) is a type of fibroblastic neoplasm that can occur in various parts of the body, with SFT of the pineal gland being exceedingly rare. We report the case of a 58-year-old male presenting with recurrent hiccups, acid reflux, and headache. Magnetic resonance imaging revealed an occupying lesion in the pineal region, suggestive of a neoplastic process. Intraoperatively, the lesion was located in the pineal region, exhibiting a grayish-red color, and was largely resected. Pathological examination confirmed the diagnosis of solitary fibrous tumor (CNS WHO Grade 1). Postoperatively, the patient was supplemented with radiotherapy, and long-term follow-up showed no signs of recurrence or metastasis.
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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.
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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.
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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.
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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.
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Solitary fibrous tumor (SFT) is a rare mesenchymal neoplasm most often arising from the pleura and rarely in extra-pleural locations, including the gastrointestinal tract. We describe two cases of a SFT presenting as submucosal colonic lesion and review the literature on this lesion. One submucosal lesion was localized in the cecum and was 10 mm in size. The second lesion presented as a 17 mm submucosal rectal lesion. Both lesions presented as well-circumscribed submucosal lesions arranged in short fascicles, blending with abundant collagenous stroma. In both cases, the spindle cells were positive for CD34, STAT6 and CD99, and molecular studies showed NAB2:STAT6 fusion supporting the diagnosis of SFT. Both patients are alive and well 10 and 5 years post-excision, respectively. In conclusion, SFT can occur in the colon as a submucosal lesion and should be included in the differential diagnosis of colonic mesenchymal lesions.
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Neoplasias del Colon , Tumores Fibrosos Solitarios , Humanos , Tumores Fibrosos Solitarios/patología , Tumores Fibrosos Solitarios/cirugía , Tumores Fibrosos Solitarios/diagnóstico , Tumores Fibrosos Solitarios/diagnóstico por imagen , Masculino , Neoplasias del Colon/patología , Neoplasias del Colon/cirugía , Neoplasias del Colon/diagnóstico , Persona de Mediana Edad , Femenino , Factor de Transcripción STAT6/análisis , Factor de Transcripción STAT6/metabolismo , Anciano , Antígenos CD34/análisis , Antígenos CD34/metabolismo , Colonoscopía , Mucosa Intestinal/patología , Mucosa Intestinal/diagnóstico por imagen , Diagnóstico Diferencial , Proteínas RepresorasRESUMEN
Solitary fibrous tumor (SFT) is a rare fibroblastic tumor with spindle cell morphology, which is characterized by a prominent branching vasculature and a NAB2-STAT6 gene rearrangement. SFT may occur in any anatomical site and may involve salivary glands, including the parotid gland. We present a young female with a primary parotid SFT diagnosed as "neoplasm-Salivary gland neoplasm of uncertain malignant potential (SUMP)" per the Milan system for reporting salivary gland cytopathology by fine-needle aspiration (FNA) with surgical pathology follow-up. Cytomorphology of SFT is diverse and overlaps with more common entities causing a diagnostic challenge. Non-diagnostic FNA results are not uncommon. Thankfully, the majority of SFTs involving the salivary gland can be identified as "neoplasm" on FNA. The Neoplasm-SUMP subcategory is considered for the majority of cases, which would warrant a diagnostic excision with clear surgical margins, which is also curative in most cases. The Neoplasm-SUMP also perfectly encompasses the neoplastic behavior of SFT, which runs on a scale from indolent to malignant.
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BACKGROUND: Solitary fibrous tumors (SFTs) manifest in various anatomical locations but are seldom encountered in the prostate. Despite their rare occurrence in this region, SFTs demonstrate a marked propensity for recurrence. This study elucidates a case of recurrent prostate SFT, previously misdiagnosed, and delineates the salient features and diagnostic criteria pertaining for SFTs. METHODS: Through a meticulous analysis of the patient's antecedent medical records and corroborative diagnostic evaluations, we hypothesized that the presenting pathology was indicative of a prostate SFT. In order to substantiate this supposition, we re-examined archival pathological specimens from the patient. The ensuing pathological assessment validated our conjecture. To address the recurrence, we conducted an open surgical procedure to excise the tumor. Subsequent postoperative pathological evaluations further corroborated the diagnosis of prostate SFT. RESULTS: Upon re-evaluation of the patient's earlier pathological specimens, we discerned that what had been previously classified as a "seminal vesicle tumor" was, in fact, a prostate SFT. During the surgical intervention, it was observed that the prostatic tumor had invaded the bladder, yet there was no seminal vesicle involvement. The tumor dimensions were approximately 7 × 5 × 4 cm, and the margin between the tumor and the surgical resection edge was less than 0.1 cm. The postoperative histological analysis confirmed the diagnosis of recurrent prostate SFT, substantiating our designation of the patient's condition as such. A year-long follow-up revealed no conspicuous signs of tumor recurrence. CONCLUSIONS: Therapeutic intervention for prostate SFT is predominantly surgical. However, given the tumor's marked predisposition for recurrence, the specific mechanisms underlying its etiology and pathogenesis remain enigmatic. Hence, a comprehensive understanding of its pathogenic and recurrent characteristics, coupled with regular postoperative surveillance, is imperative for efficacious treatment and prevention of prostate SFT.
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Fibroma , Neoplasias de la Próstata , Síndrome de Trombocitopenia Febril Grave , Tumores Fibrosos Solitarios , Masculino , Humanos , Próstata/patología , Neoplasias de la Próstata/diagnóstico , Neoplasias de la Próstata/patología , Tumores Fibrosos Solitarios/patología , Tumores Fibrosos Solitarios/cirugía , Fibroma/diagnóstico , Fibroma/cirugía , Errores DiagnósticosRESUMEN
Background: Solitary fibrous tumor (SFT) of the chest mainly arises from the pleura, but intrapulmonary SFT is rare. This study aimed to review and discuss the chest multislice computed tomography (MSCT) and magnetic resonance imaging (MRI) findings of intrapulmonary SFT and summarize existing literature on the disease in order to improve clinicians' understanding and diagnosis of this disease. Methods: The imaging findings and clinical data of 4 surgically and pathologically confirmed intrapulmonary SFT cases were retrospectively analyzed in terms of location, morphology, size, density, border, enhancement level of the lesion, and its relationship with surrounding tissue. These findings were combined with a review of 61 cases reported in the literature to characterize the features of intrapulmonary SFT. Results: A total of 65 patients with intrapulmonary SFT were reviewed, consisting of 30 females and 35 males. Of these cases, 21 had a lesion in the left lower lobe, more than in any other part of the lungs. The lesions were clear, had a quasicircular boundary, and were distinctly separated from surrounding tissue. Under plain scan, the 4 cases investigated in this study showed lesions of even density, and enhanced scanning revealed geographic enhancement in 2 cases. Of the 65 cases examined, 56 cases were benign, and the remaining 9 cases were borderline or malignant. Conclusions: The imaging findings of intrapulmonary SFT demonstrated certain features, such as lesions with a clear boundary and even density. Imaging examination is important for the diagnosis and differential diagnosis of this disease.
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Solitary fibrous tumor (SFT), originally described in the pleura, is a rare mesenchymal neoplasm characterized by a wide spectrum of clinical presentations and histopathological features. Over the years, SFTs have been reported in various anatomical locations, including soft tissues, visceral organs, and, uncommonly, the kidney. While SFTs primarily arise from the pleura, their occurrence in the kidney is an infrequent phenomenon, accounting for a minute fraction of all renal tumors. This case report presents a unique instance of a solitary fibrous tumor originating in the kidney, highlighting its clinical, radiological, and histopathological characteristics, as well as the challenges associated with accurate diagnosis and appropriate management. The rarity of such cases underscores the importance of comprehensive evaluation and awareness among clinicians and pathologists to ensure timely diagnosis and effective treatment strategies. Here, we report a case of SF of the kidney (SFT-K) located in the renal pelvis in a 39-year-old Caucasian female.
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A solitary fibrous tumor (SFT) is a rare spindle cell neoplasm in adults, usually found in the pleural and thoracic cavities. We report an interesting case of a malignant solitary fibrous tumor in a 64-year-old male who presented with a history of swelling in his right inguinal region that gradually increased in size during the past three years. Computed tomography (CT) and magnetic resonance imaging (MRI) scans of the abdomen and pelvis showed a rounded solid mass originating from the right inguinal canal suggestive of sarcoma. Elective excision of the mass was done under general anesthesia with histopathology confirming the diagnosis of high-grade spindle cell sarcoma in keeping with a malignant solitary fibrous tumor. Postoperatively, the patient had no complications and was discharged on postoperative day 4. The patient was then treated with radiotherapy. He remained free of recurrence for two years postoperatively.
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Background: Solitary fibrous tumors (SFTs) manifest in various anatomical locations but are seldom encountered in the prostate. Despite their rare occurrence in this region, SFTs demonstrate a marked propensity for recurrence. This study elucidates a case of recurrent prostate SFT, previously misdiagnosed, and delineates the salient features and diagnostic criteria pertaining for SFTs. Methods: Through a meticulous analysis of the patients antecedent medical records and corroborative diagnostic evaluations, we hypothesized that the presenting pathology was indicative of a prostate SFT. In order to substantiate this supposition, we re-examined archival pathological specimens from the patient. The ensuing pathological assessment validated our conjecture. To address the recurrence, we conducted an open surgical procedure to excise the tumor. Subsequent postoperative pathological evaluations further corroborated the diagnosis of prostate SFT. Results: Upon re-evaluation of the patients earlier pathological specimens, we discerned that what had been previously classified as a seminal vesicle tumor was, in fact, a prostate SFT. During the surgical intervention, it was observed that the prostatic tumor had invaded the bladder, yet there was no seminal vesicle involvement. The tumor dimensions were approximately 7 × 5 × 4 cm, and the margin between the tumor and the surgical resection edge was less than 0.1 cm. The postoperative histological analysis confirmed the diagnosis of recurrent prostate SFT, substantiating our designation of the patients condition as such. A year-long follow-up revealed no conspicuous signs of tumor recurrence. Conclusions: Therapeutic intervention for prostate SFT is predominantly surgical. However, given the tumors marked predisposition for recurrence, the specific mechanisms underlying its etiology and pathogenesis remain enigmatic (AU)
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Humanos , Masculino , Persona de Mediana Edad , Neoplasias de la Próstata/diagnóstico , Neoplasias de la Próstata/cirugía , Fibroma/diagnóstico , Fibroma/cirugía , Tomografía Computarizada por Rayos X , Inmunohistoquímica , ProstatectomíaRESUMEN
Solitary fibrous tumors (SFTs) are rare soft tissue tumors that can arise in the abdomen, pleura, and central nervous system, among other sites. Surgical resection is the mainstay of management, although recurrence rates remain substantial. This case describes a 73-year-old male treated surgically for both a recurrent SFT and small bowel obstruction (SBO) secondary to adhesions. The patient had undergone numerous intra-abdominal operations for malignant SFT since 1994, highlighting the importance of meticulous resection at the initial presentation of local disease.
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BACKGROUND: Solitary fibrous tumors (SFTs) are very rare spindle cell neoplasms of mesenchymal origin with largely benign course of disease. Genital SFT's can be managed providing excellent functional and psychological outcomes by timely intervention. CASE PRESENTATION: We report the largest and possibly the second only reported case of penile SFT in a 34 year male presenting with a gradually increasing perineal mass with clinically normal appearing phallus. MRI revealed a 9.8 × 3.2 cm soft tissue mass arising from left corpora cavernosae, the mass was excised en-bloc via a perineal approach under spinal anaesthesia. Histopathology revealed spindle cell tumor embedded in myxohyaline stroma along with hyalinized vascular channels demonstrating IHC positivity for CD34 and STAT6. The patient is disease free post 2 years of resection with no sexual or urinary dysfunctions. CONCLUSION: Genital SFTs, although rare, should be considered in the differential diagnosis of well-circumscribed, painless, slow growing solid masses and histopathologists must be vigilant of its malignant characteristics.
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Síndrome de Trombocitopenia Febril Grave , Tumores Fibrosos Solitarios , Humanos , Masculino , Síndrome de Trombocitopenia Febril Grave/diagnóstico , Tumores Fibrosos Solitarios/diagnóstico por imagen , Tumores Fibrosos Solitarios/cirugía , Diagnóstico Diferencial , Imagen por Resonancia Magnética , Pene/patologíaRESUMEN
Solitary fibrous tumors (SFTs) are rare vascular malignancies that are rarely reported in the upper limb, and even rarer as secondary metastasis to the small bowel. We present a case of a 39-year-old male patient, a known case of metastatic SFT, presenting with severe abdominal pain and vomiting. Computed tomography showed ileo-ileal intussusception. Subsequently, he underwent surgical resection. Histopathological examination showed high-grade sarcoma of the intestinal wall, confirming small intestinal metastasis. This constitutes one of the rare cases of SFTs metastasizing to the small bowel, specifically to the ileum. A review of the literature on similar cases is presented. There appears to be a connection between upper limb SFT and bowel metastasis according to reported literature. However, the scarcity of similar reports makes the generalizability of the conclusion limited.
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Solitary fibrous tumors (SFTs) are rare spindle cell neoplasms of mesenchymal origin that are most commonly found in the pleura, although they have also been documented in extrapleural locations. SFTs affect males and females in equal distribution, and they typically occur between the fourth and seventh decades of life. Since SFTs are usually benign and asymptomatic, the majority of them are discovered incidentally on computed tomography (CT) or magnetic resonance imaging (MRI) imaging, unless they grow to a size that causes mass effect symptoms on other organs. Nonetheless, imaging is not sufficient to diagnose an SFT, and therefore, biopsy is recommended for further analysis. Advances in immunohistochemistry and molecular diagnostics have identified CD34 and NAB2-STAT6, respectively, as the most consistent markers for SFTs. The risk of SFT metastasis can be determined through the use of a four-variable risk-stratification model developed by Demicco et al., which is based upon the risk factors of patient age, tumor size, mitotic count per 10 high-power fields, and the degree of tumor necrosis. The management of SFTs involves a wide surgical resection of the tumor while preserving surrounding organs and structures. Post-operative surveillance involves imaging the primary tumor site for up to five years due to the risk of local recurrence. At this time, neither radiation therapy nor chemotherapy after resection have yet to show benefit, and therefore, they are not currently recommended. This case report discusses the management of a 68-year-old woman who was diagnosed with a malignant extrapleural SFT in her right medial upper thigh.
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Solitary fibrous tumors are very rare in the pleura, and they are generally found incidentally. Even though they can potentially become malignant and metastasize, they have minimal clinical symptoms and can still be benign. Due to the low incidence of these tumors, there is no standard of therapy beyond surgical resection. We present an asymptomatic case of a large, rapidly expanding solitary fibrous tumor of the pleura in an elderly female.
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Solitary fibrous tumor (SFT) is a rare soft-tissue sarcoma. This nonhereditary cancer is the result of an environmental intrachromosomal gene fusion between NAB2 and STAT6 on chromosome 12, which fuses the activation domain of STAT6 with the repression domain of NAB2. Currently there is not an approved chemotherapy regimen for SFTs. The best response on available pharmaceuticals is a partial response or stable disease for several months. The purpose of this study is to investigate the potential of RNA-based therapies for the treatment of SFTs. Specifically, in vitro SFT cell models were engineered to harbor the characteristic NAB2-STAT6 fusion using the CRISPR/SpCas9 system. Cell migration as well as multiple cancer-related signaling pathways were increased in the engineered cells as compared to the fusion-absent parent cells. The SFT cell models were then used for evaluating the targeting efficacies of NAB2-STAT6 fusion-specific antisense oligonucleotides (ASOs) and CRISPR/CasRx systems. Our results showed that fusion specific ASO treatments caused a 58% reduction in expression of fusion transcripts and a 22% reduction in cell proliferation after 72 h in vitro. Similarly, the AAV2-mediated CRISPR/CasRx system led to a 59% reduction in fusion transcript expressions in vitro, and a 55% reduction in xenograft growth after 29 days ex vivo.