Abstract:Solitary fibrous tumor (SFT) is a rare neoplasm of mesenchymal origin, especially in the central nervous system (CNS). Reported herein is a case of SFT of CNS in a 63-year-old female patient who had confused mentality, without other neurological deficit. The brain MRI showed an ovoid mass in the right frontal lobe. The tumor was surgically removed grossly and totally, and the pathologic diagnosis was SFT. At 55 months after the surgery, the tumor recurred at the primary site and at an adjacent area. A second o… Show more
“…[15] (Solitary fibrous tumors of the central nervous system: clinicopathological and therapeutic considerations of 18 cases) On immunohistochemistry, SFTs are characterized by diffusive staining of CD34, vimentin, CD99 and Bcl-2, while always negative for S-100 and EMA expression, which can be easily distinguished from meningioma. [2,16,17] Negative CD34 as in our case, however, has been reported and considered as a potential indicator of poor prognosis. [5,12,18,19]…”
Rationale:
Solitary fibrous tumors of central nervous system are rare spindle-cell mesenchymal tumors. Although most are benign in nature, malignant transformation and extracranial metastasis have been reported. Up to now, only one case of CSF dissemination was described. Here we described an extremely rare case of intracranial Solitary fibrous tumors arising from the pineal region with a delayed ectopic metastasis.
Patient concerns:
A 35-year-old female presented with double vision, memory disturbance and unsteady gait was referred to our center. MRI showed an irregular mass in the pineal region.
Diagnoses:
The patient was diagnosed as pineal tumor, with unknown pathology.
Interventions:
Gross total resection was achieved and the pathologic studies confirmed a solitary fibrous tumor. Thirty-nine months later local recurrence occurred and gamma-knife radiotherapy was offered. Seven months later, MRI found a metastasis in the left temporal lobe. Surgical resection was conducted and pathological analysis revealed changes in cell morphology, counts and Ki-67 level, confirmed the diagnosis of solitary fibrous tumor/hemangiopericytoma (WHO Grade III). The patient received post-operational radiotherapy.
Outcomes:
The patient was followed up for 7 months with no signs of recurrence.
Lessons:
Here, we report an extremely rare case of primary solitary fibrous tumor of pineal region with delayed intracranial ectopic metastasis, together with literature review of metastatic solitary fibrous tumors. Strict surveillance is strongly recommended, considering the malignant potential of this seemingly benign disease entity. Complete resection of the tumor is the treatment of first choice and radiotherapy might be an effective adjuvant therapy for high grade SFT/HPCs.
“…[15] (Solitary fibrous tumors of the central nervous system: clinicopathological and therapeutic considerations of 18 cases) On immunohistochemistry, SFTs are characterized by diffusive staining of CD34, vimentin, CD99 and Bcl-2, while always negative for S-100 and EMA expression, which can be easily distinguished from meningioma. [2,16,17] Negative CD34 as in our case, however, has been reported and considered as a potential indicator of poor prognosis. [5,12,18,19]…”
Rationale:
Solitary fibrous tumors of central nervous system are rare spindle-cell mesenchymal tumors. Although most are benign in nature, malignant transformation and extracranial metastasis have been reported. Up to now, only one case of CSF dissemination was described. Here we described an extremely rare case of intracranial Solitary fibrous tumors arising from the pineal region with a delayed ectopic metastasis.
Patient concerns:
A 35-year-old female presented with double vision, memory disturbance and unsteady gait was referred to our center. MRI showed an irregular mass in the pineal region.
Diagnoses:
The patient was diagnosed as pineal tumor, with unknown pathology.
Interventions:
Gross total resection was achieved and the pathologic studies confirmed a solitary fibrous tumor. Thirty-nine months later local recurrence occurred and gamma-knife radiotherapy was offered. Seven months later, MRI found a metastasis in the left temporal lobe. Surgical resection was conducted and pathological analysis revealed changes in cell morphology, counts and Ki-67 level, confirmed the diagnosis of solitary fibrous tumor/hemangiopericytoma (WHO Grade III). The patient received post-operational radiotherapy.
Outcomes:
The patient was followed up for 7 months with no signs of recurrence.
Lessons:
Here, we report an extremely rare case of primary solitary fibrous tumor of pineal region with delayed intracranial ectopic metastasis, together with literature review of metastatic solitary fibrous tumors. Strict surveillance is strongly recommended, considering the malignant potential of this seemingly benign disease entity. Complete resection of the tumor is the treatment of first choice and radiotherapy might be an effective adjuvant therapy for high grade SFT/HPCs.
“…However, the incidence of extrapleural SFT (ESFT) has been increasing within the last two decades, and approximately more than one-third of literature in English is about ESFTs [9]. Such tumour has been observed at almost every extrapleural anatomic site, including the liver [10], kidney [11], pancreas [12], prostate [13], breast [14], central nervous system [15], reproductive system [16, 17], orbit [18], head and neck [19], pelvis [20], soft tissues in the extremities [21] and retroperitoneum [22]. However, the incidence of intravascular SFT is extremely rare [23].…”
Background
Solitary fibrous tumour (SFT) is a mesenchymal tumour of fibroblastic type, and it develops in almost any part of the human body. However, according to previous studies, the occurrence of intravascular SFTs is extremely rare.
Case presentation
We reported a case of intravascular SFT in a 67-year-old woman who has been experiencing swelling and pain in the right leg for 2 months. Computed tomography venography scan revealed a well-defined mass obstructing the inferior vena cava (IVC). Surgical resection was performed, and histopathologic and immunohistochemical results were consistent with SFT. Further, next-generation sequencing (NGS) analysis was performed, and results revealed two tumour-related gene mutations (deletion of PMS2 and variation of ESR1 [L536P]). The patient did not receive any adjuvant therapy, and no signs of tumour progression were observed during the 6-month follow-up.
Conclusion
To the best of our knowledge, this study first presented about SFT arising from the IVC and carried out an NGS analysis to validate the molecular mechanism of such condition.
“…The natural history and biological behavior of SFTs are as yet unclear, and there appears to be a variation in tumor growth rates and recurrence;1,2,4,6,7,16,22–24 however, it usually has an indolent course as reflected in the WHO classification of the tumors of the central nervous system. According to the 2007 classification criteria, SFT was classified as an independent benign pathological variation corresponding to grade I 25.…”
Section: Discussionmentioning
confidence: 99%
“…Solitary fibrous tumor (SFT) is a benign mesenchymal neoplasm of putative fibroblastic origin. They can occur anywhere in the human body but arise predominantly in the visceral pleura; SFTs in extraserosal sites, especially those involving the central nervous system, are quite infrequent 1–3. SFTs occurring in the spinal intramedullary locations are extremely rare 4–8.…”
BackgroundSolitary fibrous tumor (SFT) is a benign neoplasm arising in the soft tissue, which can occur anywhere in the body, while it is predominantly found in the visceral pleura. Spinal SFT is quite uncommon, with limited cases having been reported in the literature; especially, SFT occurring in the intramedullary site is extremely rare.Case presentationWe present a case of a 35-year-old woman presenting with progressive numbness and weakness in the legs and urinary incontinence. Magnetic resonance imaging (MRI) showed an intramedullary lesion with bright enhancement. A diagnosis of spinal hemangioblastoma was suspected, and thus a three-dimensional computed tomographic angiography reconstruction was requested, which also demonstrated an angiomatous lesion. The tumor was completely resected under neurophysiological monitoring. However, histopathological and immunohistochemical examinations revealed an SFT. No adjuvant radiotherapy or chemotherapy was scheduled. The symptoms were relieved completely, and no recurrence or progression was noted during the follow-up.ConclusionThough SFT has been considered similar to malignant hemangiopericytoma and the histological classification has always been controversial, the intramedullary location and benign behavior in the present case add to the current understandings of this extremely rare entity.
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