Materiały źródłowe

• #1 Solitary Fibrous Tumors: Symptoms, Causes & Treatment

  https://my.clevelandclinic.org/health/diseases/14945-solitary-fibrous-tumors

  Solitary fibrous tumors tend to grow slowly. Up to half of people with solitary fibrous tumors dont have symptoms. Symptoms may only occur once the tumor gets large and presses on nearby organs, bones or tissues. When they occur, symptoms vary depending on which part of your body the tumor affects. […] Signs of a solitary fibrous tumor in your chest cavity, including the pleura and lungs, include: Chronic cough. Clubbed fingers and nails (enlarged fingertips and abnormal growth of nails). Noncardiac chest pain. Shortness of breath (dyspnea). […] Signs of a solitary fibrous tumor in the orbit of your eyes can affect your vision, causing: Bulging eyes (proptosis). Double vision (diplopia). Drooping eyelid (ptosis). Eye pain. Low vision. Swollen eyelids (blepharitis). Watery eyes. […] Signs of a solitary fibrous tumor in your sinuses or nasal cavity include: Bulging eyes. Drooping eyelids. Nasal congestion. Runny nose.

• #2 FloridaHealthFinder | Solitary fibrous tumor | Health Encyclopedia | FloridaHealthFinder

  https://quality.healthfinder.fl.gov/health-encyclopedia/HIE/1/000116

  About one half of the people with this type of tumor do not show any symptoms. […] If the tumor grows to a large size and pushes on the lung, it can lead to symptoms, such as: Chest pain, Chronic cough, Shortness of breath, Clubbed appearance of the fingers. […] The outcome is expected to be good with prompt treatment. In rare cases, the tumor may return.

• #3 Solitary Fibrous Tumors – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK585038/

  Solitary fibrous tumors (SFTs) represent a rare type of mesenchymal neoplasm that can arise in various locations throughout the body, although most commonly occur in the thoracic cavity. Despite typically exhibiting a benign clinical course, a subset of SFTs may demonstrate malignant behavior, leading to local recurrence and distant metastasis. […] SFTs represent a diverse group of fibrotic mesenchymal neoplasms that are rarely malignant and have low metastatic potential. They usually follow an indolent course with an asymptomatic presentation. […] The malignant potential of SFTs is typically associated with specific clinical and histological features. These include older age, larger tumor size, increased cellularity, elevated mitotic activity (4/10 high-power fields or 2 mitoses/2 mm2), nuclear pleomorphism, tumor necrosis, and infiltrative borders.

• #4 Health Information Library | Rumah Sakit Pusat Pertamina

  https://rspp.co.id/dcontent.html?id=CON-20198292&n=Solitary%20fibrous%20tumor

  Solitary fibrous tumors tend to grow slowly. They might not cause symptoms until they grow large. Symptoms depend on where the tumor is in the body. If it’s in the lungs, symptoms might include cough and shortness of breath. […] Solitary fibrous tumor signs and symptoms can depend upon where the tumor grows. Solitary fibrous tumors often grow so slowly that they don’t cause symptoms for some time. They most often happen around the lungs, abdomen, and head and neck. […] Symptoms of a solitary fibrous tumor in or around the lungs include: Chest pain. Cough. Coughing up blood. Swelling of fingers or toes, called clubbing. Shortness of breath. Swelling in the lower legs. […] Symptoms of a solitary fibrous tumor in the abdomen include: Abdominal pain. Constipation. Involuntary urine leaking. Painful urination. Urinating frequently at night. Vomiting. […] Symptoms of a solitary fibrous tumor in the head and neck include: Bulging eyes. Frequent tearing. […] Solitary fibrous tumors that happen in other places in the body may cause painless lumps or masses.

• #5 Solitary fibrous tumor – Wikipedia

  https://en.wikipedia.org/wiki/Solitary_fibrous_tumor

  About 80% of pleural SFTs originate in the visceral pleura, while 20% arise from parietal pleura. […] Although they are often very large tumors (up to 40 cm. in diameter), over half are asymptomatic at diagnosis. […] Some SFTs are associated with the paraneoplastic DoegePotter syndrome, which is caused by tumor production of IGF-2. […] Prognosis in benign SFTs is excellent. About 8% will recur after first resection, with the recurrence usually cured after additional surgery. […] The prognosis in malignant SFTs is much more guarded. Approximately 63% of patients will have a recurrence of their tumor, of which more than half will succumb to disease progression within two years.

• #6 Solitary fibrous tumor – Davanzo – Translational Gastroenterology and Hepatology

  https://tgh.amegroups.org/article/view/4708/html

  SFT tumors may be found in almost any site of the body with intra-thoracic being the most common location followed by intra-abdominal. […] The majority, between 50-80%, of thoracic SFT will present as asymptomatic masses discovered incidentally on chest imaging. Those that do present with symptoms present with nonspecific chest complaints such as chest pain, dyspnea or cough. […] Intra-abdominal SFT are generally asymptomatic until they reach large enough size to cause mass effect on other organs. […] On rare occasions SFT can present with paraneoplastic syndromes, the most commonly described being non-islet cell hypoglycemia. […] Tumor size at presentation is highly variable and is typically associated with location. The median size among several studies is noted between 7-10 cm with a range from anywhere from 1 to 40 cm.

• #7 Solitary Fibrous Tumour – Rare Cancers Australia

  https://www.rarecancers.org.au/knowledgebase/cancer-types/solitary-fibrous-tumour/

  Solitary fibrous tumours (SFTs), also known as hemangiopericytomas, are a rare type of tumour that generally develops in mesenchymal or fibrous tissue in the body. SFTs can be found anywhere in the body, and are generally benign. However, in rare cases SFTs can become malignant. SFTs are generally diagnosed equally amongst the genders, and tend to be diagnosed between the ages of 50-70. However, anyone can develop this disease. […] Symptoms of a pleuropulmonary SFT may include: Persistent cough. Dyspnea. Chest pain. Haemoptysis. Inflammation of the lungs. Paraneoplastic syndromes. […] Symptoms of an intraabdominal SFT may include: Abdominal mass. Abdominal pain. Unexplained weight loss/loss of appetite. Changes in urinary habits, potentially including: Dysuria. Urinary retention. Hydronephrosis. Polyuria. Incontinence. Nausea and/or vomiting. Paraneoplastic syndromes. Constipation. […] Symptoms of a dural SFT may include: Focal deficits. Seizures. Increased intracranial pressure (ICP), which carries its own set of symptoms: Blurred vision. Decreased alertness. Nausea and/or vomiting. Behavioural changes. Weakness/difficulties moving or talking. Fatigue. Confusion. Hypertension. Shallow breathing. Headache. Paraneoplastic syndromes. […] Symptoms of an extracranial SFT in the head and neck region may include: Mass on the eyelid. Excess tear production (if around the eye). Proptosis. An obstruction of the sinus and/or nasal passages. Painless mass in affected area. Paraneoplastic syndromes. […] Symptoms of a soft tissue SFT may include: Painless mass in affected region. Nerve pain (if tumour grows onto a nerve). Paraneoplastic syndromes. […] Symptoms of an SFT in the spinal cord may include: Back pain. Radicular pain. Numbness, tingling, and/or weakness in the legs. Difficulty walking. Back pain that is worse at night. […] Not everyone with the symptoms above will have cancer, but see your general practitioner (GP) if you are concerned.

• #8 Solitary fibrous tumour | Sarcoma UK

  https://sarcoma.org.uk/about-sarcoma/what-is-sarcoma/types-of-sarcoma/solitary-fibrous-tumour/

  Symptoms of SFT can vary depending on the size and location of your tumour. Before receiving a diagnosis of SFT, you may experience all, some, or none of these symptoms. […] A lump or swelling in the soft tissue. This lump is often slow-growing and painless. […] You may experience: Coughing, Shortness of breath, Chest pain. […] You may experience: A swollen abdomen, An inability to completely empty your bladder when urinating, Constipation, A feeling of being very full even after only eating a small amount of food. […] You may experience: A blocked nose, Changes in voice, Nosebleeds. […] Some larger SFTs are known to release a hormone which can cause a condition called Doege-Potter syndrome. This syndrome can cause signs and symptoms such as: Low blood sugar, which can result in headaches, tiredness, neurological problems, and an increased heart rate. Rarely, increased growth in certain parts of the body due to a condition called acromegaly.

• #8 Solitary fibrous tumour | Sarcoma UK

  https://sarcoma.org.uk/about-sarcoma/what-is-sarcoma/types-of-sarcoma/solitary-fibrous-tumour/

  After treating a previous tumour, SFT can come back in the same place. This is known as a local recurrence. […] A recurrence of SFT may be accompanied by cancer in other parts of the body. This is called metastasis or secondary cancer. Some people are diagnosed with sarcoma because their tumour has spread before the main tumour is found. SFT patients may develop secondary cancers in the lungs. […] Secondary cancers may also appear in the liver and bones. If you have advanced cancer, your treatment may involve surgery, radiotherapy, or other therapies. Doctors will evaluate your treatment on a case-by-case basis.

• #9 Solitary Fibrous Tumors: Symptoms, Causes & Treatment

  https://my.clevelandclinic.org/health/diseases/14945-solitary-fibrous-tumors

  Signs of a solitary fibrous tumor in your brain include: Delirium. Dizziness. Gait disorders. Headaches. Hearing loss. One-sided paralysis (hemiplegia). […] Signs of a solitary fibrous tumor in your spinal cord include: Back pain. Neck pain. Neuropathy or neuropathic (nerve) pain. Numbness. Shoulder pain. Urinary incontinence. […] Signs of a solitary fibrous tumor in your liver include: Bloated stomach. Discomfort or malaise (generally not feeling well). Fatigue. […] Signs of a solitary fibrous tumor in your thyroid include: Difficulty swallowing (dysphagia). Hoarseness. Lump or mass in your neck. Shortness of breath.

• #10 Solitary Fibrous Tumor – EyeWiki

  https://eyewiki.org/Solitary_Fibrous_Tumor

  Solitary fibrous tumors of the orbit are rare spindle-shaped neoplasias that typically present as benign orbital masses in middle-aged adults and are typically cured with complete surgical excision. […] Solitary fibrous tumors of the orbit are typically benign slow growing lesions. As such, patients typically present with months to years of progressive proptosis. […] Patients present with proptosis and possibly ptosis, eyelid swelling, tearing, diplopia or decreased vision. Malignant infiltrating lesions can sometimes cause pain.

• #11 Solitary Fibrous Tumour – SFA

  https://curesarcoma.org/sarcoma-subtypes/solitary-fibrous-tumour/

  Most tumors present as slow-growing, painless masses. Abdominopelvic tumors may present with distention (swelling), constipation, urinary retention, or early satiety (feeling full quickly), whereas head and neck SFTs may present with nasal obstruction, voice changes, or bleeding. […] Large SFTs may cause paraneoplastic syndromes such as Doege-Potter syndrome, with the induction of severe hypoglycemia (low blood sugar) or (more rarely) acromegaloid changes (enlargement of hands, feet, and face) due to tumor production of IGF2 (insulin-like growth factor 2). […] Recurrence (distant or local) occurs in 10-30% of SFTs, with 10-40% of recurrences reported after 5 years and rare recurrences seen after 15 years. TERT promoter mutations are more common in tumors with aggressive features, but they may also be seen in low-risk SFT. Various single clinical or histological features have been reported to correlate with metastatic or local recurrence potential in large series, including high mitotic count (> 2 mitoses/mm2, equating to > 4 mitoses per 10 high-power fields of 0.5 mm in diameter and 0.2 mm2 in area), tumor size, necrosis, patient age, tumor cellularity and nuclear pleomorphism, and tumor site. However, individual studies contradict one another regarding which specific features are important.

• #12

  https://braintumourresearch.org/pages/types-of-brain-tumours-haemangiopericytoma?srsltid=AfmBOoq7zt3BWqgQfRa4TIPSwWuKDVYJy0Q1Qb854kxCj_KfdCHnHCEU

  The symptoms will depend upon exactly where the tumour is in the brain, and hence which parts of brain function are affected. Symptoms are likely to include one of more of the following: Changes in vision, such as seeing double or blurriness […] Headaches that worsen with time […] Hearing loss or ringing in the ears […] Memory loss […] Loss of smell […] Seizures […] Weakness in the arms or legs.

• #13 Malignant Hepatic Solitary Fibrous Tumor

  https://www.e-jlc.org/journal/view.php?number=432

  Hepatic solitary fibrous tumors (SFTs) are mostly benign and rare because of information regarding the clinical symptoms, treatment, and prognosis of their malignant forms is currently lacking. […] Only a few cases of malignant hepatic SFT have been reported, and right upper quadrant (RUQ) pain, weight loss, and loss of consciousness are some of the first symptoms to appear. […] Malignant hepatic SFTs are extremely rare and according to a literature review, only been five cases of malignant hepatic SFT have been reported. […] Unlike benign SFTs, which frequently have no symptoms, malignant SFTs showed RUQ pain as its first symptom, and there were various cases with systemic symptoms, including weight loss and loss of consciousness. […] Usually, the prognosis of malignant SFT is poor.

• #14 Pathology Outlines – Solitary fibrous tumor

  https://www.pathologyoutlines.com/topic/softtissuesft.html

  Symptoms associated with anatomic location […] Somatic soft tissue tumors present as slow growing, painless mass […] Abdominopelvic tumors produce symptoms due to organ impingement […] Pleural tumors often discovered incidentally but could grow exophytically into lungs […] Paraneoplastic syndrome (Doege-Potter syndrome) extremely rare; due to IGF2 production by tumor.

• #15 Solitary Fibrous Tumor – DoveMed

  https://www.dovemed.com/diseases-conditions/solitary-fibrous-tumor?trendmd_shared=0

  Most small tumors are asymptomatic, though the larger ones may compress the surrounding structures and cause related signs and symptoms. […] Small tumors usually do not cause any symptoms. But, occasionally they may become painful, if they compress surrounding structure. […] Solitary Fibrous Tumors may occur as slowly enlarging painless mass. The following symptoms may be seen: Increased skin temperature over the tumor, if the tumor is visible over the skin; Telangiectasia (abnormal dilation of red, blue, or purple superficial blood vessels) over the tumor; Unilateral dilated veins (varicose veins) in the region of the tumor; Abnormal vibration of pulse (pulsation), or bruit (abnormal sounds heard on auscultation) over the tumor; Restriction of joint movement if the tumor is close to a joint.

• #15 Solitary Fibrous Tumor – DoveMed

  https://www.dovemed.com/diseases-conditions/solitary-fibrous-tumor?trendmd_shared=0

  Rarely, Solitary Fibrous Tumors of Soft Tissue, especially if large, may cause hypoglycemia. […] If the tumor involves the pleura, it is usually asymptomatic. However, sometimes, these tumors can cause symptoms such as cough or shortness of breath. […] The signs and symptoms of hypoglycemia include confusion, altered mental status, sweating, dizziness, cold hands, etc.

• #16 Solitary Fibrous Tumors – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK585038/

  Tumors with such features are known to recur and metastasize. […] SFTs commonly metastasize through hematogenous dissemination, most frequently to the lungs, followed by the intrabdominal organs. […] Metastatic disease, mainly spread to the lungs, is often the primary cause of mortality associated with SFTs. […] Despite tumor size often being considered a negative prognostic factor, SFTs can grow large without exhibiting aggressive behavior. […] Paraneoplastic syndromes, although rare in SFTs, can present significant clinical challenges. […] Hypertrophic osteoarthropathy (HOA), also called Pierre-Marie Bamberg syndrome, is observed in less than 10% of cases (usually pleural) and often manifests as finger clubbing, hypertrophic skin changes, arthralgia, and increased periosteal activity. […] Clubbing may be asymptomatic or associated with pain, typically affecting distal phalanges. […] The effects of these syndromes typically diminish once the causative tumor is surgically removed.

• #17 A Comprehensive Review on Solitary Fibrous Tumor: New Insights for New Horizons

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8230482/

  Clinically, SFTs present as a well-defined mass, which is more silent in primary pleural locations than extra-pleural primary sites. […] Few patients, less than 10%, suffer paraneoplastic syndromes such as hypertrophic osteoarthropathy (HOA), seen in cases of pleural SFT. […] The overexpression of vascular endothelial growth factor (VEGF) is deemed to be the main underlying pathogenic mechanism. […] The diagnosis of HOA is based on the combination of clubbing and an increase in periosteal activity detected in plain X-ray. […] The risk of metastatic spread can be as high as 3545%, or even greater, in series with a longer follow-up period. […] Recurrences beyond 10 years were seen in up to 10% of the SFT patients. […] Even with a longer follow-up, the relapse-free survival can be as low as 18% at 20 years.

• #17 A Comprehensive Review on Solitary Fibrous Tumor: New Insights for New Horizons

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8230482/

  Solitary fibrous tumor (SFT) is a malignant condition that exhibits different clinical behaviors ranging from low to high aggressive SFT, with dedifferentiated SFT (DD-SFT) being the fastest-growing subtype. […] Even when surgery alone provides curation rates above 60%, recurrences do occur in a fraction of patients where surgery is unable to provide disease control. […] The risk for metastasis is up to 3545%, or even greater, in series with a longer follow-up. […] Over the last few decades, advances in immunohistochemistry and molecular diagnostics identified STAT6 nuclear protein expression and the NAB2STAT6 fusion gene as more precise tools for SFT diagnosis. […] Recent evidence taken from retrospective series and from two prospective phase II clinical trials showed that antiangiogenics are active and their sequential use from first line should be considered, except for dedifferentiated SFT for which chemotherapy is the best option.

• #18

  https://link.springer.com/article/10.1007/s10555-024-10204-8

  In certain circumstances, paraneoplastic syndromes may be observed. Approximately 10% of SFT patients may exhibit a „Pierre Marie-Bmberger syndrome,” characterized by symptoms of hypertrophic osteoarthropathy resulting from VEGF overexpression. Additionally, around 5% of SFT patients may present with a „Doege-Potter Syndrome,” a refractory hypoglycemic syndrome caused by the overproduction of IGF2.

• #18

  https://link.springer.com/article/10.1007/s10555-024-10204-8

  Solitary fibrous tumor (SFT) is a sporadic disease with an incidence rate of 12 per million. Typically, SFT grows slowly and hence patients can be asymptomatic. It can originate in various anatomical sites, with the pleura being the most common location. […] The clinical presentation of SFTs depends on the primary tumor location. CNS SFTs can present with symptoms related to increased intracranial pressure, such as headaches, nausea, and visual disturbances. Depending on their specific location within the CNS, these tumors may cause focal neurological deficits, including weakness, sensory changes, or seizures. In contrast, most non-CNS SFTs are found incidentally on routine physical examination. Tumor sizes can vary greatly, spanning from 1 to 40 cm. Symptoms tend to manifest when the tumor reaches a median size of approximately 5-8 cm, causing compression of nearby organs or tissues. While most SFT remains localized, a minority can metastasize to common sites such as the lungs, liver, and bones.

• #19 Diagnostics and Treatment of Extrameningeal Solitary Fibrous Tumors

  https://www.mdpi.com/2072-6694/15/24/5854

  In some cases, systemic symptoms such as arthralgia, hypertrophic osteoarthropathy, or clubbing occur. […] Hypoglycemia is associated with weight loss, fatigue, night sweats, or unsteady gait. […] Furthermore, it is considered a poor prognostic indicator because, in approximately 70% of cases, tumors present malignant behavior. […] However, all systemic symptoms could resolve after resection of the mass.

• #20 Solitary fibrous tumor of the dura | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/solitary-fibrous-tumour-of-the-dura?lang=us

  Solitary fibrous tumors are composed of spindle cells thought to be of mesenchymal origin. They usually occur in middle-aged individuals and symptoms depend on the size of the mass and its location. […] Clinical presentation is nonspecific and identical to other dural masses (e.g. meningioma). Symptoms depend on the size of the mass and its location. Headache is the most common presenting complaint. […] Rarely, solitary fibrous tumors result in non-islet cell tumor hypoglycemia (NICTH) as a result of secretion of insulin-like growth factor-2 (IGF-2). Rarer still is acromegaly. […] Surgical resection, if complete, is usually curative. Recurrence rates are variably reported as low to relatively high; up to 50% in one series. If incomplete or recurrence occurs, then radiotherapy may be employed.

• #21

  https://www.sydneysarcomaunit.com.au/solitary-fibrous-tumour

  Solitary Fibrous Tumours grow slowly over many years and may not cause symptoms until they have grown to a large size. […] Most tumours present as a slow growing mass that do not cause symptoms. As the tumour increases in size it may cause symptoms to occur, these depend on the location of the tumour in the body and are variable.

• #22 Solitary fibrous tumor | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/solitary-fibrous-tumour?lang=us

  Solitary fibrous tumors are a rare neoplasm of mesenchymal origin that comprise less than 2% of all soft tissue tumors ranging from indolent tumors to more aggressive masses. […] The clinical presentation of solitary fibrous tumors is usually due to local mass effect and therefore will depend on the location and size of the tumor. Tumors in the head and neck tend, therefore, to present at a smaller size, whereas pleural and abdominal tumors later. […] Rarely solitary fibrous tumors result in paraneoplastic non-islet cell tumor hypoglycemia (NICTH) known as Doege-Potter syndrome which results from the secretion of insulin-like growth factor-2 (IGF-2). […] In contrast, high risk tumors tend to be larger at the time of diagnosis (10 cm), more commonly encountered in older individuals and have the expected histological features (increased cellularity, increased mitotic activity, nuclear pleomorphism, etc.) as well as necrosis and local infiltration. These have a poorer prognosis, especially if incompletely resected. […] Metastasis rates can be as high as ~35% (range 25-45%) with late recurrence common (up to 10%). […] Optimal adjuvant therapy for this group is unknown, and close-interval follow-up is advised because there is an increased incidence of local recurrence.

• #23 Solitary fibrous tumor: a clinicopathological study of 110 cases and proposed risk assessment model | Modern Pathology

  https://www.nature.com/articles/modpathol201283

  Solitary fibrous tumors are categorized as intermediate biological potential with a low risk of metastasis and relatively indolent course under the 2002 WHO Classification. […] However, the clinical behavior of individual tumors is notoriously difficult to predict. […] Several histopathological criteria have been reported to be useful for determining malignancy in solitary fibrous tumor. These include increased tumor size, mitotic count, cellularity, presence of hemorrhage/necrosis, nuclear pleomorphism, and presence of sharply demarcated anaplastic/poorly differentiated foci. […] In our series, the strongest predictors of time to tumor metastasis and disease-specific death were patient age at presentation, tumor size, and mitotic index. […] Necrosis was also predictive of time to metastasis but not time to disease-specific death.

• #24

  https://link.springer.com/article/10.1007/s00109-019-01815-8

  We wanted to evaluate whether SFTs progress to malignant transformation under TERT promoter mutations. The results were not available in 18 cases. In the remaining 73 SFT cases, only the -124CT TERT promoter mutation was detected in seven cases (10%) and was strongly associated with malignant SFTs (P=0.003) and the presence of necrosis (P=0.036). However, the association between TERT promoter mutation and clinically aggressive behavior, which includes recurrence and/or metastasis, was not strong enough to show statistical significance (P=0.205). All seven instances of TERT promoter mutations were discovered in older patients (≥56 years old). […] We also found statistically significant association between TP53 immunohistochemical positivity and malignant SFTs (P=0.006) and clinical history of recurrence and/or metastasis (P=0.006). In addition, we found TP53 mutations in 41% (7/17) of the malignant SFTs in our cohort by molecular analysis.

• #25 Solitary fibrous tumor: a clinicopathological study of 110 cases and proposed risk assessment model | Modern Pathology

  https://www.nature.com/articles/modpathol201283

  Primary tumors that went on to metastasize occurred in older patients, were larger, had increased mitotic activity, and were more likely to have necrosis. […] In patients with observed local recurrence or metastasis, median times to recurrence and metastasis were 84 months and 36 months, respectively. […] Overall 5- and 10-year metastasis-free rates were 74 and 55%, respectively. […] In our study, 50% of metastases occurred after 36 months, but could present up to 19 years after initial resection. […] The 5-year metastasis-free rates in moderate and high-risk groups were 77 and 15%, respectively, while 10-year metastasis-free rates were 64 and 0%. […] No patients in the low-risk group developed metastasis or died of disease, over a median follow-up of 50 months.

• #26 Solitary fibrous tumor – Davanzo – Translational Gastroenterology and Hepatology

  https://tgh.amegroups.org/article/view/4708/html

  Seventy percent of these tumors will exhibit malignant behavior and presence of non-islet cell hypoglycemia is an overall poor prognostic indicator. […] Malignant SFT tumors, however, like other STS have a less durable response with up to 63% recurring even after complete resection. […] While the behavior of most tumors is benign but potentially locally aggressive, a significant fraction of patients will have tumors that demonstrate malignant behavior. The biologic behavior of individual SFTs is hard to predict, and even tumors considered histologically benign may aggressively recur. […] Multiple factors have been associated with survival. Gold et al. demonstrated recurrent tumors, macro- or microscopically positive resection margins, tumor size 10 cm, 4 mitoses/10 HPF, increased nuclear pleomorphism, increased cellularity and presence of malignant component all to be poor prognostic factors.

• #27 Rapid growth of a solitary fibrous tumor of the pleura after slow long-term progression: a case report | Journal of Cardiothoracic Surgery | Full Text

  https://cardiothoracicsurgery.biomedcentral.com/articles/10.1186/s13019-024-03142-7

  Solitary fibrous tumors (SFTs) of the pleura are usually benign. We present a case of SFT of the pleura which grew rapidly after slow long-term progression. […] Patients with SFTs present with dyspnea, chest pain, cough, clubbing of fingers, and hypoglycemia, although most cases are asymptomatic and detected incidentally. […] However, infrequently, they can grow rapidly. […] SFTs require surgical intervention because of their potential for rapid growth. […] As the tumor grows, SFT can cause respirocirculatory failure by compressing the lungs, diaphragm, or heart. […] Surgical resection should be considered for suspected SFTs with a tendency to increase in size. […] SFTs have the potential for rapid growth after more than 5 years. […] In conclusion, SFTs require surgical treatment due to their potential for rapid growth.

• #28 Clinical outcomes in central nervous system solitary-fibrous tumor/hemangiopericytoma: a STROBE-compliant single-center analysis | World Journal of Surgical Oncology | Full Text

  https://wjso.biomedcentral.com/articles/10.1186/s12957-022-02619-w

  Solitary fibrous tumor (SFT) and hemangiopericytoma (HPC) are rare mesenchymal tumors in the central nervous system with a high tendency to relapse, having a significant impact on quality of life (QoL). […] The PFS and OS were 29 months (range 4 to 96) months and 38 (range, 4 to 125) months. […] The median EQ5D-3L tariff of the patients with or without progression were 0.617 (95% CI: 0.4700.756) and 0.939 (95% CI: 0.7720.977), respectively. The EQ5D-3L tariff significantly decreased in patients whose disease progressed (p=0.002). […] Consequently, QoL in intracranial SFT/HPC patients significantly declined when the tumor relapsed. Intracranial patients with tumor recurrence and metastasis were intended to suffer body pain and stay in negative emotion frequently. […] Several prognosis factors were detected for disease progression, involving LD (5.0cm and 5.0cm), EOR, adjuvant therapy, and WHO grade.

• #29

  https://journals.lww.com/ajsp/fulltext/2009/09000/expanding_the_spectrum_of_malignant_progression_in.7.aspx

  Dedifferentiation is a well recognized, if sometimes controversial, form of tumor progression in certain types of soft tissue and bone sarcoma, and confers a worse prognosis when compared with the low-grade counterpart. […] Our results demonstrate that dedifferentiation in SFT, comparable with that in other low grade/intermediate soft-tissue tumors, poses a higher risk of tumor recurrence and/or metastasis, most notably in large and deep-seated tumors. […] Similar to other dedifferentiated sarcomas, abrupt transition between low grade and high-grade areas is typically observed with loss of CD34 positivity.

• #30

  https://journals.lww.com/md-journal/fulltext/2016/01120/postoperative_radiotherapy_for_the_treatment_of.34.aspx

  Solitary fibrous tumor of the pelvic is an uncommon neoplasm with nonspecific symptoms. […] The patient was initially treated with surgery, followed by postoperative dimensional conformal intensity modulated radiation therapy (dynamic MLC VRIAN 23EX Linac, inversely optimized by the Eclipse system) to provide a radical cure for residual tumor. […] In this case, there were no signs of recurrence after six and a half years of further follow-up, indicating that postoperation radiotherapy may be an effective treatment for SFT with malignant transformation in pelvic. […] Although nearly all SFTs have low malignant potential, malignancy can occur, especially if they grow to a large size or in the case of repeated recurrence. […] The main treatment for SFT is surgery. […] The diagnosis was malignant SFT.

• #31 Solitary fibrous tumor – Augusta HealthSearchClose SearchSearch IconSearch IconClose Search IconMobile Menu IconMobile Menu Close IconInstagramFacebookTwitterYoutube

  https://www.augustahealth.com/disease/solitary-fibrous-tumor/

  Solitary fibrous tumors tend to grow slowly and may not cause signs and symptoms until they become very large. […] Certain drugs target the vascular endothelial growth factor (VEGF) and other tyrosine kinase signaling pathways. These pathways disrupt the blood supply to the tumor, and have recently begun to be used to treat advanced solitary fibrous tumors to slow their progression.

• #32 Malignant solitary fibrous tumor in the central nervous system treated with surgery, radiotherapy and anlotinib: A case report

  https://www.wjgnet.com/2307-8960/full/v10/i2/631.htm

  No standardized treatment guideline is available for malignant intracranial SFTs. The main treatments are surgical resection and postoperative radiotherapy. […] Despite the combination of surgery and adjuvant radiotherapy, the control rates of malignant SFT have been disappointing. […] The patient was diagnosed with malignant SFT. […] The patient underwent left craniotomy under general anesthesia, and the mass was resected. […] MRI (1.5 mo after surgery) showed a solid mass on the cut margin. SFT after subtotal resection has a high risk of local recurrence, and adjuvant radiation may be beneficial in some cases. […] The patient received 60 Gy and 30 fractions of intensity modulated radiotherapy. […] Our patient showed that radiotherapy resulted in excellent local control of intracranial malignant SFT. […] Follow-up MRI showed that the tumor had not progressed 1.5 years after surgery. […] There is currently no standard treatment regimen for malignant SFT of the CNS. There is no effective targeted drug that can improve the prognosis of malignant intracranial SFT.

• #33 Comprehensive analysis reveals potential therapeutic targets and an integrated risk stratification model for solitary fibrous tumors | Nature Communications

  https://www.nature.com/articles/s41467-023-43249-4

  Solitary fibrous tumors (SFTs) are rare mesenchymal tumors with unpredictable evolution and with a recurrence or metastasis rate of 10-40%. […] Despite an indolent course, aggressive behavior in the form of local recurrence or distant metastasis may still occur in 10-40% of the SFT patients. […] SFTs comprise a histological spectrum and have an unpredictable evolution, and some SFTs can still progress despite having a clearly benign appearance. […] Our findings hold promise for advancing therapeutic strategies and refining risk prediction in SFTs. […] High density of CD68+ macrophages, CD163+ macrophages, and HLA-DPB1+ cells infiltrated in SFTs tissues was associated with shorter PFS of the patients. […] In addition, we found that two SFT cases showed both IDH1 p.R132S mutation and high PD-L1 expression. […] Our integrated risk model has demonstrated superior accuracy in identifying high-risk primary non-CNS and CNS SFTs with NTM, and showed better performance compared to established models such as the WHO classification, mDemicco model, and G-score.

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