Outside the Thorax: Doege–Potter Syndrome Presenting as a Retroperitoneal Abdominal Mass

Objective. We present a case of refractory hypoglycemia, weight loss, and retroperitoneal solitary fibrous tumor. Case report. A 68-year-old female presented with symptomatic hypoglycemia, weight loss, and abdominal mass identified on CT scan of the abdomen. Blood work during symptomatic hypoglycemia was consistent with an IGF-2-producing tumor. The abdominal mass pathology was consistent with solitary fibrous tumor surrounding the adrenal gland, and resection resulted in complete resolution of hypoglycemia. Discussion. Understanding the biochemical mechanisms behind glucose regulation is necessary to diagnose and adequately treat Doege–Potter syndrome, a paraneoplastic syndrome observed in patients with solitary fibrous tumors. Solitary fibrous tumors can be characterized by specific histologic and immunohistochemical studies. Conclusion. This report describes the clinical workup of a patient presenting with hypoglycemia and a retroperitoneal tumor. This case is unique because of its presentation with severe, refractory hypoglycemia and the tumor’s location in the retroperitoneum, given the majority of solitary fibrous tumors are found in the lungs originating from the pleura.

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Solitary fibrous tumor of the greater omentum: case report and review of literature

Surgical Case Reports ◽

10.1186/s40792-021-01176-w ◽

2021 ◽

Vol 7 (1) ◽

Author(s):

Karim M. Eltawil ◽

Carly Whalen ◽

Bryce Knapp

Keyword(s):

Solitary Fibrous Tumor ◽

Management Strategy ◽

Abdominal Mass ◽

Lower Abdominal Pain ◽

Greater Omentum ◽

Team Approach ◽

Review Of Literature ◽

Case Presentation ◽

Multidisciplinary Team Approach ◽

Fibrous Tumor

Abstract Background Solitary fibrous tumor (SFT) is a rare neoplasm of mesenchymal origin occurring most often in the visceral pleura, however, it has been described in almost every anatomic location of the human body. While most SFTs have a benign behavior, they can potentially be locally aggressive and demonstrate a malignant behavior. Case presentation A 63 year-old male patient presented with lower abdominal pain and nausea and was noted on CT to have a large, heterogeneous lower abdominal mass with no evidence of metastatic disease. A surgical resection was performed and the mass appeared to be connected to the greater omentum with a vascular pedicle. It was not invading any intra-abdominal or pelvic organs. Pathology revealed an SFT of omental origin. The mitotic count was less than 4 per 10 high-power fields and all pathologic characteristics did not meet the criteria for a malignant SFT. Conclusions We report an extremely rare case of SFT originating from the greater omentum. A multidisciplinary team approach was followed to plan the patient’s management strategy.

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Malignant Solitary Fibrous Tumor of the Right Cerebellum: A Case Report

Case Reports in Neurology ◽

10.1159/000510844 ◽

2021 ◽

Vol 13 (1) ◽

pp. 259-266

Author(s):

Ye-Tao Zhu ◽

Yang Liu ◽

Li-Gang Chen ◽

Da-Ping Song

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Solitary Fibrous Tumor ◽

Immunohistochemical Staining ◽

Biological Behavior ◽

Resonance Imaging ◽

Solitary Fibrous Tumors ◽

The Central Nervous System ◽

Fibrous Tumor ◽

The Right

Solitary fibrous tumor is a very rare mesenchymal tumor that occurs mostly in the pleura, and there are few reported cases of a presence in the central nervous system, particularly in the cerebellum. In 2016, the WHO classified solitary fibrous tumors into grade I. In this article, we present a case of malignant solitary fibrous tumor recurring 8 years after surgery in a 63-year-old male. Magnetic resonance imaging showed low to intermediate mixed signal intensity on T1W1. Immunohistochemical staining positivity for Vimentin, CD99, CD34 and Bcl-2, it is consistent with the immunohistochemical characteristics of solitary fibrous tumor. We resected the patient’s tumor, and the patient was followed up for 3 months with no signs of recurrence. Solitary fibrous tumors are very rare in the central nervous system. Immunohistochemical staining positivity for CD34 and Bcl-2 is strongly expressed in most solitary fibrous tumor. Surgical resection is the preferred treatment. Due to the small number of cases, the biological behavior and prognosis of this tumor need to be further explored.

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A Solitary Fibrous Tumor of the Pleura Revealed by Hiccups

Case Reports in Medicine ◽

10.1155/2011/574319 ◽

2011 ◽

Vol 2011 ◽

pp. 1-3 ◽

Cited By ~ 2

Author(s):

A. Chafik ◽

M. Alaoui ◽

A. Benjelloune ◽

Y. Qamouss

Keyword(s):

Solitary Fibrous Tumor ◽

Similar Case ◽

Clinical Presentation ◽

Malignant Potential ◽

Pleural Tumor ◽

Solitary Fibrous Tumors ◽

Progressive Dyspnea ◽

Fibrous Tumor

Solitary fibrous tumors of the pleura are rare and benign primary localized tumors; they possess a malignant potential and thus should be excised. We report a case of a 43-year-old woman, who had suffered for 5 years from right basithoracic pain associated with progressive dyspnea and persistent hiccups during the last 6 months. We have not found any similar case in the literature. Further testing after excision by thoracotomy revealed a solitary fibrous pleural tumor. A brief discussion of the clinical presentation and incidence of these tumors is included.

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Solitary Fibrous Tumor of the Larynx: A Case Report and Review of the Literature

Archives of Pathology & Laboratory Medicine ◽

10.5858/2006-130-213-sftotl ◽

2006 ◽

Vol 130 (2) ◽

pp. 213-216 ◽

Cited By ~ 5

Author(s):

Jorge E. Dotto ◽

William Ahrens ◽

David J. Lesnik ◽

Diane Kowalski ◽

Clarence Sasaki ◽

...

Keyword(s):

Case Report ◽

Solitary Fibrous Tumor ◽

Review Of The Literature ◽

Solitary Fibrous Tumors ◽

Subsequent Investigation ◽

Mesenchymal Neoplasms ◽

Fibrous Tumor

Abstract Solitary fibrous tumors are relatively rare mesenchymal neoplasms that were originally described as pleural- or peritoneal-based lesions. Although they were considered a form of mesothelioma, subsequent investigation failed to reveal mesothelial differentiation. Characterization of their histologic and immunohistochemical features, as well as identification in a multitude of nonmesothelial-based locations has further served to distinguish these lesions from the more diffuse and aggressive mesothelioma. Reports of solitary fibrous tumor in the larynx are extremely rare. We report a case of solitary fibrous tumor of the larynx in a 38-year-old man.

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Solitary Fibrous Tumor of the Orbit With a t(9;22)(q31;p13)

Archives of Pathology & Laboratory Medicine ◽

10.5858/2000-124-0756-sftoto ◽

2000 ◽

Vol 124 (5) ◽

pp. 756-758

Author(s):

Dean M. Havlik ◽

Denise A. Farnath ◽

Therese Bocklage

Keyword(s):

Solitary Fibrous Tumor ◽

Genetic Information ◽

Solitary Fibrous Tumors ◽

Fibrous Tumor

Abstract Solitary fibrous tumors are well-described neoplasms found predominantly in the subpleural region but also in many other body sites. They generally behave in a benign fashion, although a few cases that exhibit a malignant course have been reported. Genetic information on solitary fibrous tumors is sparse. This case illustrates a previously unreported finding of a tumor-specific t(9;22)(q31;p13) in a solitary fibrous tumor of the orbit of a 58-year-old man.

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Case Report: A Malignant Liver and Thoracic Solitary Fibrous Tumor: A 10-Year Journey From the Brain to the Liver and the Spine

Frontiers in Surgery ◽

10.3389/fsurg.2020.570582 ◽

2020 ◽

Vol 7 ◽

Author(s):

Min Mao ◽

Lei Zhou ◽

Chaojun Huang ◽

Xudong Yan ◽

Shuo Hu ◽

...

Keyword(s):

Solitary Fibrous Tumor ◽

Disease Free Survival ◽

Liver Lesion ◽

Management Plan ◽

Gross Total Resection ◽

Thoracic Vertebrae ◽

Total Resection ◽

Meningeal Tumor ◽

Solitary Fibrous Tumors ◽

Fibrous Tumor

Solitary fibrous tumors are rare neoplasms that originate from mesenchymal tissues and have been found to occur in any site, including the spine and liver. Although most of solitary fibrous tumors have benign features, only 10–20% are malignant and prone to metastasis. No previous reports have described the malignant and metastatic Solitary fibrous tumor arising in both of the liver and thoracic vertebrae. In this article, we present the case of a 60-year-old woman who underwent gross total resection of a meningeal tumor in 2007. She presented 10 years later with a thoracic vertebral mass that caused relentless pain and a lesion in the right lobe of liver. She underwent marginal excision of the T3 tumor with T2-4 pedicular screw fixation in March 2017, then right hemi-hepatectomy was performed to remove the liver lesion in June 2017. Both of the lesions were confirmed to be a metastatic and malignant tumor after surgery. The literature lacks randomized controlled trials and large studies that define the natural history of malignant solitary fibrous tumors and recommendations of precise management plan for the disease. However, the best choice for treatment is gross total resection, which probably provide the optimal treatment to achieve long-term disease-free survival.

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Solitary Fibrous Tumor of the Parapharyngeal Space: A Case Report and Review of the Literature

Ear Nose & Throat Journal ◽

10.1177/014556139607501011 ◽

1996 ◽

Vol 75 (10) ◽

pp. 681-684 ◽

Cited By ~ 15

Author(s):

Kunal Gangopadhyay ◽

Khalid Taibah ◽

M. Babu Manohar ◽

Hala Kfoury

Keyword(s):

Case Report ◽

Head And Neck ◽

Solitary Fibrous Tumor ◽

Spindle Cell ◽

Parapharyngeal Space ◽

English Literature ◽

Review Of The Literature ◽

Solitary Fibrous Tumors ◽

Rare Location ◽

Fibrous Tumor

Solitary fibrous tumors are uncommon spindle cell neoplasms generally associated with serosal surfaces, especially the pleura. Recently, these tumors have been documented in a number of extrapleural sites including the head and neck. So far only two cases of parapharyngeal solitary fibrous tumor have been reported in the English literature. Rare location of an uncommon lesion often gives rise to difficulty in diagnosis or to misdiagnosis. In both the previously reported cases, as well as in our case, the diagnosis of solitary fibrous tumor was not made until the excised tumor was subjected to histopathology and immunohistochemistry.

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Solitary Fibrous Tumors and So-Called Hemangiopericytoma

Sarcoma ◽

10.1155/2012/690251 ◽

2012 ◽

Vol 2012 ◽

pp. 1-6 ◽

Cited By ~ 42

Author(s):

Nicolas Penel ◽

Eric Yaovi Amela ◽

Gauthier Decanter ◽

Yves-Marie Robin ◽

Perrine Marec-Berard

Keyword(s):

Solitary Fibrous Tumor ◽

Targeted Therapies ◽

Standard Treatment ◽

Tumor Biology ◽

Special Focus ◽

Solitary Fibrous Tumors ◽

Systemic Treatments ◽

Localized Disease ◽

Definition Of ◽

Fibrous Tumor

We have reviewed the literature data regarding the spectrum of tumors including solitary fibrous tumor and hemangiopericytoma with special focus on definition of the disease, discussion of the criteria for malignancy, and the key elements of standard treatment of localized disease. We have discussed the emerging concepts on the tumor biology and the different systemic treatments (chemotherapy and molecular-targeted therapies).

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Solitary Fibrous Tumor of the Kidney. A Propos of a Case

Urologia Journal ◽

10.1177/039156030907600214 ◽

2009 ◽

Vol 76 (2) ◽

pp. 112-114 ◽

Cited By ~ 1

Author(s):

M. Marzi ◽

M. D'Alpaos ◽

P. Piras ◽

A. Paiusco ◽

M.S. Minervini ◽

...

Keyword(s):

Solitary Fibrous Tumor ◽

Renal Pelvis ◽

Spindle Cell ◽

Left Kidney ◽

Clinical Behavior ◽

Spindle Cell Neoplasm ◽

Solitary Fibrous Tumors ◽

Genital Apparatus ◽

Cell Neoplasm ◽

Fibrous Tumor

Solitary Fibrous Tumors (SFT) are rare spindle cell neoplasm that typically originate from the pleura. However, cases of the SFT are described with origin in other organs, including the urinary and genital apparatus. Within the kidney, except from the renal pelvis, only 19 cases of SFT are described and such rarity of localization makes the histogenesis and the prognosis of the tumor rather unknown. We report the case of a 72-year-old lady who attended our Unit for a mass that was clinically palpable at the level of the left hemiabdomen. The tomodensitographic test indicated a 19cm-diameter mass of likely pertinence of the middle bystender of the left kidney. She had undergone left radical nephrectomy. The histological examination highlighted a solitary fibrous tumor (SFT): the presence of hypercellularity, of cellular pleiomorphism and of a high number of mitosis has led to a histological diagnosis of malignancy for the neoplasm analyzed. The SFT are of rare clinical comparison: this does not allow for a deep knowledge of the lesion histogenesis and prognosis; moreover, the clinical behavior should be more precisely defined.

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