Retroperitoneal Solitary Fibrous Tumor Research Articles

Postoperative Morbidity After Radical Resection of Retroperitoneal Solitary Fibrous Tumor.

BackgroundThis study aimed to investigate the clinicopathological characteristics of retroperitoneal solitary fibrous tumor (RSFT) and the safety of radical resection.MethodsA retrospective analysis was conducted on the data of 32 RSFT patients who received surgery with curative intent from February 2011 and June 2021.ResultsThis cohort included 16 (50%) male and 16 (50%) female patients, with the median age of 52 (29 to 72) years. Tumor burden ranged from 3 to 25 (median, 10) cm. Seven patients received arterial embolization before surgery. 15 (47%) patients received radiotherapy, nine (28%) of which received preoperative radiotherapy. Most of the patients (91%) achieved complete resection with median bleeding of 400 (20 to 5,000) ml. Nine (28%) patients received packed red blood cell (RBC) transfusion, with a median of 5 (2 to 10) U. All patients had the five-year progression-free survival rate and the overall survival rate of 75.8% and 80.0%, respectively. 11 (34%) patients were found with adverse events, and four (12%) patients were found with serious postoperative complications (Clavien-Dindo ≥3), of which one (3.1%) patient died after surgery. The univariate analysis found that tumor burden (p = 0.022), packed RBC transfusion (p = 0.001) and postoperative hospital stays (0.027) were correlated with overall morbidity. The multivariate analysis found packed RBC transfusion as an independent risk factor for postoperative morbidity (HR 381.652, 95% CI, 1.597–91213.029, p = 0.033).ConclusionRSFT was confirmed as an uncommon, slow-growing and recurring tumor, with acceptable postoperative morbidity and mortality after surgical resection.

Think Outside the Thorax: Doege-Potter Syndrome Presenting as a Retroperitoneal Mass

Introduction: Non-islet cell tumor hypoglycemia (NICTH) is a rare paraneoplastic syndrome, occurring less commonly than insulinoma. These tumors produce high molecular weight insulin-like growth factor-2 or “big IGF-2”, resulting in hypoglycemia: big IGF-2 decreases glycogenolysis, gluconeogenesis, and glucagon release as well as increases glucose uptake by adipocytes and skeletal muscle. NICTH specifically associated with a solitary fibrous tumor (SFT) is known as Doege-Potter syndrome. Doege-Potter syndrome is seen most with thoracic SFT’s. This case study details the diagnosis and management of Doege-Potter syndrome due to retroperitoneal SFT.Case Description: Our patient is a 68-year-old, white female who initially presented to an outside facility with a 3-week history of episodic neuroglycopenic symptoms temporarily relieved with eating. She reported night sweats but no weight change or fever. Medical history was notable only for hypertension and GERD. She had no personal or family history of diabetes. She was transferred to our facility for further evaluation of an 18.6 cm multilobular mass seen just inferior to the liver on abdominal CT. Whipple’s triad was confirmed with venous glucose sampling while inpatient. C-peptide, free/total insulin, and pro-insulin were collected during permissive hypoglycemia and were low. Morning cortisol and TSH were unremarkable. Additionally, sulfonylurea screen and insulin antibodies were negative. Ultrasound-guided biopsy of the lesion was positive for CD34 and STAT6, consistent with SFT. Refractory hypoglycemia was treated with D5W infusion until surgical resection of the retroperitoneal mass, after which, she had complete resolution of her hypoglycemia.Discussion: This patient’s serum IGF-2 was within normal limits, consistent with reports of feedback inhibition exerted by increased levels of big-IGF-2. Therefore, the IGF-2:IGF-1 ratio is used to determine increased serum levels of big IGF-2, but this test is not widely available. SFT’s can recur within months to years of resection, necessitating surveillance. Recurrence is also more common with extrathoracic tumors. Surgical pathology noted tumor dimensions of 22 cm and 2270 g; mitotic rate was 1 per 10 hpf. Features typical of malignancy include large size (>15 cm) and mitotic rate >4 mitoses per hpf. This patient’s follow-up CT abdomen at 6 weeks did not show any findings to suggest recurrent or metastatic disease.

Outcome of patients with primary retroperitoneal solitary fibrous sarcoma.

To describe the clinicopathological features of primary retroperitoneal solitary fibrous tumor (RSFT) and define the prognostic factors. The comprehensive data of 35 primary RSFT patients who got curative surgery at a tertiary cancer center from April 2004 to October 2018 were retrospectively analyzed. Male patients outnumbered female patients (19 vs. 16), with the age ranging from 19 to 73years (median, 51years). 7 (20%) patients had tumors located in special parts, including three in kidney, one in renal pelvis, one in bladder, one in prostate, and one in mesentery. Tumor sizes ranged from 2.5 to 25cm (median, 9cm). Microscopic negative margin was reached in 33 (94.3%) cases. 13 (37.1%) were classified as atypical/malignant, while 22 (62.9%) were benign. Concomitant organ excision was performed on 11 (31.4%) patients, with kidney (n = 5) being the most frequent organ. Multifocality was only found in 4 (11.4%) cases. The majority of the patients (31, 88.6%) did not get adjuvant treatment. The median follow-up time was 46months (range 4-153months). The 5-year DSS rate and DFS rate were 100% and 63.6%, respectively. In univariate analysis, tumor size ≥ 10cm (P = 0.002) and atypical/malignant pathology (P = 0.024) were associated with decreased DFS. Multivariate analysis revealed that tumor size was the only independent prognostic factor for DFS (HR 6.03, 95% CI 1.18-30.77, P = 0.031). RSFT is uncommon, slow-growing, and recrudescent tumors. Large tumor size and malignant pathology are associated with decreased DFS. Tumor size ≥ 10cm independently predicts shortened DFS.

Integrally calcified solitary fibrous tumor in the retroperitoneum: a case report and review of the literature.

Solitary fibrous tumor (SFT) is a rare stromal neoplasm and usually occurs in the thoracic cavity. We here report a case of retroperitoneal SFT with prominent calcification. A 64-year-old man presented with an incidentally detected retroperitoneal mass in the right upper abdomen. Imaging tests indicated an integrally calcified mass. The lesion was observed for 2 years and laparoscopically resected according to the patient’s wish. Microscopically, the mass was mostly occupied by calcification and proliferous spindle cells were scattered with positive CD34 expression. We diagnosed morphologically benign SFT and the patient remained disease-free 1 year after the excision. There has been no report of such integrally calcified SFT. Retroperitoneal SFT is difficult to make a preoperative diagnosis, and careful follow-up after the excision is recommended because morphological malignancy does not always correspond to clinical malignancy.

Retroperitoneal solitary fibrous tumor: surgery as first line therapy.

BackgroundSolitary fibrous tumors (SFT) of the retroperitoneum are rare spindle cell neoplasms, with a paucity of data on treatment outcomes. We hypothesized that surgical excision offered acceptable outcomes in SFTs.MethodsThe National Cancer Database (NCDB) was used to identify patients with SFT from 2004 to 2011. Primary outcome measures were 30 day mortality and overall survival. Descriptive analyses were performed. Furthermore, a systematic review of published literature was conducted after creating a pre-specified search strategy.ResultsOf 51 patients in the NCDB, 58.8 % (n = 30) were males, with a median age 60 years (IQR 49–72 years). Median tumor size was 16 cm (IQR 11–21 cm). Surgical resection was performed in 92.2 % (n = 47) with 63.8 % (n = 30) having a margin negative resection. Peri-operative mortality was 2.1 % (n = 1). Of survival outcomes available for 18 patients, the median OS was 51.1 months. From the systematic review, we identified 8 studies, with 24 patients. Median age and tumor size was similar to the NCDB [47.5 years (IQR 39–66.5 years), 12 cm (IQR 7–17 cm)]. Majority [91.7 % (n = 22)] underwent surgical excision alone while one received adjuvant chemotherapy and none received radiation. After median follow up of 54 months (IQR 28–144 months), 79.2 % (n = 19) were alive without disease. Three patients (12.5 %) died of disease, one was alive with disease and one was lost to follow up. Recurrence was reported in 16.7 % (n = 4) of patients.ConclusionComplete surgical excision is a viable treatment modality for retroperitoneal SFT leading to long term survival. Low recurrence rates would argue against the need for routine adjuvant radiation or chemotherapy.

A rare cause of benign retroperitoneal mass mimicking adnexial malignancy in a postmenopausal woman: retroperitoneal fibroma

© 2014 Akpak et al; licensee Herbert Publications Ltd. This is an Open Access article distributed under the terms of Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0). This permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Correspondence We read with great interest the article by G Bassi et al., [1] in which the authors presented a case named “An incidental retroperitoneal solitary fibrous tumor”. They mentioned about the clinicopathological characteristics of retroperitoneal solitary fibrous tumor and analyse the diagnosis of retroperitoneal fibrosis. Unfortunately, there are only a few cases of retroperitoneal fibroma reported in the literature. When we search “retroperitoneal fibroma” in PubMed we found only five literatures in english about this rare condition. The total number of reported cases has been fewer than thirteen. Therefore there is limited information about the retroperitoneal fibromas. Fibromas are stromal tumours composed of spindle, oval or round collagen-producing cells that resemble fibroblasts [2]. Herein, we present a patient with pelvic retroperitoneal fibroma which was confirmed histopathologically following surgery. Imaging findings of the tumor mimicked an adnexial mass preoperatively. A 54-year-old postmenopausal multiparous woman presented us with a complaint of lower abdominal pain, dyspareunia and urinary retention for 3 years. The patient had a history of total abdominal hysterectomy operation 16 years ago. A solid, movable mass was noted in the middle and left lower quadrant of abdomen by abdominopelvic examination. Transvaginal ultrasonography revealed a smooth surfaced solid tumoral mass with calcific degeneration measuring 86x82x88 mm located in the middle region near the vagina cuff, occupying the left adnexial area (Figure 1A). We did not see ureteral obstruction or ectasia of renal pelvis by abdominal ultrasonography. Laboratory examinations including tumor markers (AFP, CA 125, CEA, CA199, CA 15-3) were unremarkable. Pelvic magnetic resonance imaging demonstrated about 8x8 cm in size pelvic solid mass with heterogeneous internal structure, showing no significant enhancement, located in the middle and left adnexial region (Figures 1B and 1C). However, a malignant lesion could not be ruled out. One month after the initial examination, laparotomy *Correspondence: yasamaster@gmail.com

Non-islet cell induced hypoglycemia by “big-IGF-2” in a patient with retroperitoneal solitary fibrous tumor and a papillary thyroid carcinoma: An unusual association

Non-islet cell induced hypoglycemia by “big-IGF-2” in a patient with retroperitoneal solitary fibrous tumor and a papillary thyroid carcinoma: An unusual association

Doege-Potter syndrome presenting with hypoinsulinemic hypoglycemia in a patient with a malignant extrapleural solitary fibrous tumor: a case report

IntroductionDoege-Potter syndrome is a paraneoplastic syndrome characterized by non-islet cell tumor hypoglycemia secondary to a solitary fibrous tumor. This tumor causes hypoglycemia by the secretion of a prohormone form of insulin-like growth factor II. We describe the diagnosis and management of Doege-Potter syndrome and the use of transarterial chemoembolization in a patient with a malignant extrapleural solitary fibrous tumor.Case presentationOur patient was a 64-year-old Caucasian woman who initially presented with urinary incontinence and was found to have a 14.5×9.0×9.0cm retroperitoneal solitary fibrous tumor compressing her bladder. Her tumor was surgically resected but recurred with multiple hepatic metastatic lesions. The hepatic metastases progressed despite systemic chemotherapy and treatment with doxorubicin transarterial chemoembolization. Her course was complicated by the development of recurrent fasting hypoglycemia, most likely secondary to Doege-Potter syndrome. Her hypoglycemia was managed with corticosteroid therapy and frequent scheduled nutrient intake overnight.ConclusionsThe rarity of hepatic solitary fibrous tumors and consequent lack of controlled trials make this report significant in that it describes the diagnostic approach to Doege-Potter syndrome, describes our experience with the use of doxorubicin transarterial chemoembolization, and presents management options for tumor-associated hypoglycemia in the case of extensive disease not amenable to surgical resection.

Retroperitoneal solitary fibrous tumor of the pelvis with pollakiuria: a case report

BackgroundSolitary fibrous tumor (SFT) is rare soft tissue tumor, and it occurs most commonly in the pleura. Retroperitoneal SFT is generally found by palpable mass or abdominal distention. Here we report a case of SFT presenting pollakiuria.Case presentationA 64-year-old man was referred to our hospital for pollakiuria. Contrasted-enhanced computed tomography revealed a heterogeneously enhanced pelvic tumor of approximately 10 × 8 × 7 cm. Invasion of the surrounding organs, distal metastasis, and lymph node swelling were absent. Therefore, surgical resection was performed. The resected specimen was a 13 × 8 × 5.5-cm encapsulated elastic hard tumor weighing 420 g. Histologically, the tumor consisted of oval or spindle cells growing in a random manner in a collagenous matrix. Immunohistochemically, the specimen was positive for CD34, bcl-2, as well as vimentin and negative for c-kit. On the basis of these findings, a retroperitoneal solitary fibrous tumor (SFT) of the pelvis was diagnosed.ConclusionSurgery is the primary treatment for SFT, and pathologically negative margins are important for good prognosis.

A case of the large retroperitoneal solitary fibrous tumor

A rare autopsy case of the extremely large retroperitoneal solitary fibrous tumor is reported. A 52-year-old female with a huge abdominal distention was found dead at home. She showed remarkable emaciation. The autopsy revealed a huge retroperitoneal tumor weighing 11.9kg (36×30×20cm in size), which occupied the entire intraperitoneal cavity. Histologically, the tumor consisted of spindle parenchymal cells with fibrous tissues. Immunohistochemically, CD34 was positively stained, whereas S-100, smooth muscle actin, and factor VIII were negative. Her cause of death was diagnosed as emaciation due to the compression of the entire intestine by the tumor. This is a rare case of the extremely large retroperitoneal solitary fibrous tumor, which caused the occasional intestinal obstruction. This disease should be considered in the differential diagnosis of retroperitoneal large tumors that cause accidental deaths in forensic autopsies.

Solitary Fibrous Tumor of the Retroperitoneum: Case Report and Review of the Literature

Solitary fibrous tumor (SFT) is a rare mesenchymal tumor first described in 1931 by Klemperer and Rabin [1]. Most commonly occurring in the pleura, every localization has been reported, including the liver, soft tissue, orbit, intracerebral, retroperitoneal, etc. Herein, we describe a typical case of retroperitoneal SFT, its clinical, radiological, and histological features and an exhaustive review of the literature on this rare tumor.

腎細胞癌，腎血管筋脂肪腫に合併した副腎Solitary fibrous tumorの1例

Solitary fibrous tumor (SFT) is a neoplasm of pleura and its occurrence in the retroperitoneal space is rare. We report a case of SFT of the adrenal gland associated with ipsilateral renal cell carcinoma (RCC) and angiomyolipoma (AML). A 48-year-old woman was referred to our hospital for a left renal AML. Computed tomography (CT) in our hospital showed a left adrenal mass (25 x 20 mm). Because the adrenal tumor was nonfunctioning, she was followed at outpatient clinic. Four years later, CT showed an increase in the left adrenal tumor size (42 x 30 mm) and a left RCC. Left adrenectomy and partial nephrectomy for RCC and AML were simultaneously performed. Histological examination revealed adrenal SFT and clear cell carcinoma and AML of the kidney. We present a brief review on histological characteristics of retroperitoneal SFT and its occurrence in the adrenal grand region.

Retroperitoneal Solitary Fibrous Tumor-Induced Hypoglycemia Associated with High Molecular Weight Insulin-Like Growth Factor II

A man, aged 65 years, presented with frequent episodes of hypoglycemia and unconsciousness. Hypoglycemia was accompanied by undetectable serum insulin and C-peptide levels and a high serum insulin-like growth factor (IGF)-II level. He was found to have a retroperitoneal solitary fibrous tumor. He underwent successful resection of the tumor and had no hypoglycemic episodes after the operation. Immunohistochemical analysis revealed positive immunostaining for IGF-II in tumor cells. The presence of the high-molecular-weight form of IGF-II in the patient's serum was confirmed by immunoblotting, which suggests that his hypoglycemia was due to an increase in the plasma level of IGF-II secreted by the tumor.

Primary solitary fibrous tumor (SFT) in the retroperitoneum

Background Solitary fibrous tumor (SFT) is an infrequent but distinct neoplasm, which generally arises from submesothelial connective tissue in the pleura. SFT is rarely recognized in extrathoracic sites, and histologically identical conditions have also been reported in the retroperitoneum, although their pathophysiology has not been extensively investigated. Methods We present four cases of primary SFT in the retroperitoneum, and review 37 similar cases in the previous literature. Results About 40% of patients were asymptomatic, and 19.2% and 15.4% presented with an abdominal mass and urinary symptoms, respectively. The tumor size ranged between 2 and 26 (mean 9.1) cm. Sixty-three percent of tumors showed nonspecific development with haphazard distribution of bland short spindle or polygonal cells with or without collagenous bundles and stromal hyalinization. In 22.0%, hemangiopericytomatous appearance was seen. About 15% of cases showed histologically malignant characteristics. The tumor cells were immunoreactive for vimentin in all cases, CD34 in 91% and Bcl-2 in 86%. All tumors were excised, and in 85.4% of cases, tumors did not recur postoperatively for 6 to 48 months. No significant difference was found between the recurrence rate of histologically benign and malignant cases. Cases positive for both CD34 and Bcl-2 had no recurrence. Conclusions The identification of SFT in the retroperitoneum is of importance because histopathological indicators of malignancy are not necessarily associated with clinical malignant potential in many cases of retroperitoneal SFT. Retroperitoneal SFT showing typical pathological features with expression of CD34 and Bcl-2 is associated with a favorable outcome following excision.