scholarly journals Doege-Potter Syndrome with a Benign Solitary Fibrous Tumor: A Case Report and Literature Review

2021 ◽  
pp. 470-476
Author(s):  
Turab Mohammed ◽  
Gonca Ozcan ◽  
Ayesha S. Siddique ◽  
Ronald N. Araneta III ◽  
Dennis E. Slater ◽  
...  
Keyword(s):  
Solitary Fibrous Tumor ◽  
Gene Fusion ◽  
Islet Cell Tumor ◽  
Hypertrophic Osteoarthropathy ◽  
Complete Surgical Resection ◽  
Giant Tumor ◽  
Unclear Etiology ◽  
Fibrous Tumor ◽  
Tumor Hypoglycemia

Doege-Potter syndrome is a rare paraneoplastic syndrome that is often diagnosed incidentally during the workup of hypoglycemia of unclear etiology. It is characterized by a non-islet cell tumor hypoglycemia secondary to excessive production of partially processed IGF-II hormone from a solitary fibrous tumor (SFT). Often these tumors are intrathoracic, benign, and asymptomatic. Occasionally they present as a paraneoplastic event; hypertrophic osteoarthropathy in Pierre-Marie-Bamberger syndrome and hypoglycemia in Doege-Potter syndrome. The NAB2-STAT6 gene fusion is the hallmark of the SFT. Complete surgical resection of the tumor often results in resolution of symptoms and cure in most cases. Here we present the case of an 83-year-old non-diabetic female with recurrent syncopal events who was diagnosed with the Doege-Potter syndrome secondary to a SFT of pleura. Her tumor was positive for NAB2-STAT6 gene fusion on RT-PCR. Following the resection of the giant tumor mass, she became symptom-free within 24 h, and has remained asymptomatic at 4 months follow-up.

Solitary Fibrous Tumor Associated With Non-Islet Cell Tumor Hypoglycemia

2007 ◽  
Vol 84 (1) ◽  
pp. 292-294 ◽  
Author(s):  
Kotaro Kameyama ◽  
Norihito Okumura ◽  
Yujiro Kokado ◽  
Kentaroh Miyoshi ◽  
Tomoaki Matsuoka ◽  
...  
Keyword(s):  
Solitary Fibrous Tumor ◽  
Islet Cell ◽  
Islet Cell Tumor ◽  
Cell Tumor ◽  
Fibrous Tumor ◽  
Tumor Hypoglycemia

scholarly journals Solitary fibrous tumor of the pleura: 3 case reports

2015 ◽  
Vol 61 (3) ◽  
pp. 207-208 ◽  
Author(s):  
Elias Amorim
Keyword(s):  
Solitary Fibrous Tumor ◽  
Case Reports ◽  
Benign Lesion ◽  
Complete Resection ◽  
The Other ◽  
Posterolateral Thoracotomy ◽  
Pertinent Literature ◽  
Complete Surgical Resection ◽  
Fibrous Tumor

Summary Introduction: solitary fibrous tumor of the pleura (SFTP) is a rare tumor arising from mesenchymatous cells in submesothelial pleural tissue which, unlike mesothelioma, is not related to asbestos or smoking. Methods: report of four patients who underwent surgical treatment for giant SFTP and review of the pertinent literature. Results: of the four patients operated, two presented symptoms including cough, chest pain and feeling of compression, whereas the other two subjects were asymptomatic. All patients underwent complete surgical resection by wide posterolateral thoracotomy, and surgical specimens removed with minimum bleeding. None of the cases required complementary lobectomy or segmentectomy. All tumors were histologically benign. Conclusion: complete resection of the lesion is the treatment of choice in all SFTP cases. Prognosis of the benign lesion is excellent, although close follow-up is necessary. In the rarer, more aggressive forms, treatment may be complemented by adjunctive chemotherapy or radiotherapy, the benefits of which have yet to be confirmed.

scholarly journals Late Onset of Non-islet Cell Tumor Hypoglycemia Managed via Multidisciplinary Treatment in a Patient with a Solitary Fibrous Tumor

2018 ◽  
Vol 57 (16) ◽  
pp. 2431-2436 ◽  
Author(s):  
Satoshi Takeuchi ◽  
Tomohiro Goda ◽  
Jun Taguchi ◽  
Yuichi Douhata ◽  
Rio Honma ◽  
...  
Keyword(s):  
Solitary Fibrous Tumor ◽  
Islet Cell ◽  
Late Onset ◽  
Islet Cell Tumor ◽  
Multidisciplinary Treatment ◽  
Cell Tumor ◽  
Fibrous Tumor ◽  
Tumor Hypoglycemia

scholarly journals Intra-Abdominal Solitary Fibrous Tumor secretes high molecular weight IGF-II, causing Hypoglycemia

2020 ◽  
Vol 5 (5) ◽  
Keyword(s):  
Molecular Weight ◽  
Solitary Fibrous Tumor ◽  
Islet Cell ◽  
Spindle Cell ◽  
Islet Cell Tumor ◽  
Cell Tumor ◽  
Spindle Cell Neoplasm ◽  
Cell Neoplasm ◽  
Fibrous Tumor ◽  
Tumor Hypoglycemia

Hypoglycemia is a common event that has many well-known causes. Nevertheless, Non-islet cell tumor hypoglycemia (NICTH) is not one of them. As a matter of fact, NICTH is a rare but serious paraneoplastic syndrome. Solitary Fibrous tumor (SFT) is a rare spindle cell neoplasm derived from mesenchymal cells that in just 5% of cases releases “The Big” Insulin-like Growth Factor (IGF) II leading to hypoglycemic events [1].

scholarly journals Non-islet cell tumor hypoglycemia caused by intrathoracic solitary fibrous tumor: a case report

2016 ◽  
Vol 11 (1) ◽  
Author(s):  
Masahiro Kitada ◽  
Shunsuke Yasuda ◽  
Nana Takahashi ◽  
Satoshi Okazaki ◽  
Kei Ishibashi ◽  
...  
Keyword(s):  
Case Report ◽  
Solitary Fibrous Tumor ◽  
Islet Cell ◽  
Islet Cell Tumor ◽  
Cell Tumor ◽  
Fibrous Tumor ◽  
Tumor Hypoglycemia

scholarly journals Non-Islet Cell Tumor Hypoglycemia Caused by Recurrent Pelvic Solitary Fibrous Tumor

Cureus ◽  
2021 ◽  
Author(s):  
Diana Pinho dos Santos ◽  
Rui Correia ◽  
Adelino Carragoso ◽  
Carlos Casimiro ◽  
Ana Lemos
Keyword(s):  
Solitary Fibrous Tumor ◽  
Islet Cell ◽  
Islet Cell Tumor ◽  
Cell Tumor ◽  
Fibrous Tumor ◽  
Tumor Hypoglycemia

scholarly journals A Case of Non-islet Cell Tumor Hypoglycemia with Malignant Solitary Fibrous Tumor of the Pleura

Haigan ◽  
2013 ◽  
Vol 53 (1) ◽  
pp. 59-63 ◽  
Author(s):  
Youko Kataoka ◽  
Mayumi Oshio ◽  
Tomoyuki Igarashi ◽  
Makoto Motoishi ◽  
Satoru Sawai ◽  
...  
Keyword(s):  
Solitary Fibrous Tumor ◽  
Islet Cell ◽  
Islet Cell Tumor ◽  
Cell Tumor ◽  
Fibrous Tumor ◽  
Tumor Hypoglycemia

scholarly journals A Novel NFIX-STAT6 Gene Fusion in Solitary Fibrous Tumor: A Case Report

2021 ◽  
Vol 22 (14) ◽  
pp. 7514
Author(s):  
David S. Moura ◽  
Juan Díaz-Martín ◽  
Silvia Bagué ◽  
Ruth Orellana-Fernandez ◽  
Ana Sebio ◽  
...  
Keyword(s):  
Solitary Fibrous Tumor ◽  
Gene Fusion ◽  
Wide Spectrum ◽  
Bioinformatic Analysis ◽  
Fusion Partner ◽  
Rna Seq ◽  
Genomic Location ◽  
Nuclear Factor I ◽  
New Gene ◽  
Fibrous Tumor

Solitary fibrous tumor is a rare subtype of soft-tissue sarcoma with a wide spectrum of histopathological features and clinical behaviors, ranging from mildly to highly aggressive tumors. The defining genetic driver alteration is the gene fusion NAB2–STAT6, resulting from a paracentric inversion within chromosome 12q, and involving several different exons in each gene. STAT6 (signal transducer and activator of transcription 6) nuclear immunostaining and/or the identification of NAB2–STAT6 gene fusion is required for the diagnostic confirmation of solitary fibrous tumor. In the present study, a new gene fusion consisting of Nuclear Factor I X (NFIX), mapping to 19p13.2 and STAT6, mapping to 12q13.3 was identified by targeted RNA-Seq in a 74-year-old female patient diagnosed with a deep-seated solitary fibrous tumor in the pelvis. Histopathologically, the neoplasm did not display nuclear pleomorphism or tumor necrosis and had a low proliferative index. A total of 378 unique reads spanning the NFIXexon8–STAT6exon2 breakpoint with 55 different start sites were detected in the bioinformatic analysis, which represented 59.5% of the reads intersecting the genomic location on either side of the breakpoint. Targeted RNA-Seq results were validated by RT-PCR/ Sanger sequencing. The identification of a new gene fusion partner for STAT6 in solitary fibrous tumor opens intriguing new hypotheses to refine the role of STAT6 in the sarcomatogenesis of this entity.

Pazopanib-associated secondary adrenal insufficiency in a patient with malignant solitary fibrous tumor

2021 ◽  
pp. 107815522110160
Author(s):  
Muhammed Muhiddin Er ◽  
Murat Araz ◽  
Meryem Karabacak ◽  
Muzaffer Uğraklı ◽  
Melek Karakurt Eryılmaz ◽  
...  
Keyword(s):  
Adrenal Insufficiency ◽  
Solitary Fibrous Tumor ◽  
Single Case ◽  
Soft Tissue Sarcomas ◽  
Pituitary Hormones ◽  
Secondary Adrenal Insufficiency ◽  
Primary Adrenal Insufficiency ◽  
Hypoglycemic Attack ◽  
Fibrous Tumor

Introduction Pazopanib is an agent that is being successfully used in soft tissue sarcomas. Some endocrine side effects may develop during pazopanib treatment. Here, we presented a case diagnosed with secondary adrenal insufficiency while being investigated for etiology of hypoglycemia which developed after pazopanib. Case report A 69-year-old male patient was operated in June 2019 due to a lung mass 26 × 18 × 10 cm in size. Pathological diagnosis revealed a solitary fibrous tumor with malignant behavior. The patient received three lines of chemotherapy. After pazopanib treatment, a hypoglycemic attack was reported. Management and outcome: Blood cortisol and ACTH (Adrenocorticotropic hormone) levels were not increased at the time of the hypoglycemic attack, and levels of other pituitary hormones were found to be normal. Electrolyte levels were in normal range. Since the counteracting hormone did not reach a sufficient level, it was considered secondary adrenal insufficiency. Hypoglycemic attacks did not occur during follow-up while taking steroid therapy and pazopanib. Discussion A single case of primary adrenal insufficiency has been reported in the literature. We here present a case who developed hypoglycemia after pazopanib and was diagnosed with drug-associated secondary adrenal insufficiency. When hypoglycemia develops during pazopanib treatment, we must be aware of adrenal insufficiency.

Sign in / Sign up

Export Citation Format

Share Document