Doege-Potter Syndrome Research Articles

Solitary Fibrous Tumour of Liver Presenting as Doege–Potter Syndrome – A Case Report

Background: Doege–Potter syndrome is a rare syndrome which is defined as Solitary fibrous tumour (SFT) that is associated with NICTH (non-islet cell tumour hypoglycaemia). Limited case reports are available on Solitary fibrous tumour of liver presenting as Doege–Potter syndrome in published medical literature. Case report: A 60-year-old female presented with episodic weakness and incoherent behaviour since 3 months and during that episode her serum glucose were low which corrected on glucose administration and abdominal distension for 1 month. Abdominal lump was noted in right hypochondrium arising from liver. On investigation- fasting serum glucose levels, fasting insulin levels and fasting C peptide levels were low and serum IGFII levels were elevated. CT abdomen revealed heterogeneously enhancing solitary lesion of 23 x 18 x 14 cm arising from segment 5 ,6, 7, 8 of liver. AFP, CA19-9 levels were within normal range. Liver biopsy shows spindle cell tumour of fibroblastic origin. Anatomical right hepatectomy with MHV preservation was done. Postoperative course was uneventful with symptomatic improvement, serum glucose levels normalised, and patient was discharged on 8th POD. Discussion: Solitary fibrous tumours are uncommon benign or malignant mesenchymal cell tumours which originate from visceral pleura, pelvis, retroperitoneum and liver. Doege–Potter syndrome which is a rare paraneoplastic manifestation of Solitary fibrous tumour, when there is secretion of prohormone - insulin-like growth factor II (IGF-II), causing hypo insulinemic hypoglycaemia and suppressed serum insulin, C peptide and elevated IGFII/IGFI ratio . Definitive treatment is complete tumour resection.

CD34-negative solitary fibrous tumour of the broad ligament with doege-potter syndrome: a case report

Solitary fibrous tumours (SFT) are rare fibroblastic mesenchymal tumours that can occur in various anatomical sites but are extremely rare in the female genital tract. The broad ligament itself is a rare location for primary tumours and SFT in the broad ligament has only been described twice before in the literature. We report on a case of CD34-negative SFT in a 61-year-old female who presented with Doege-Potter syndrome. This case highlights the unusual presentation and location, atypical CD34 expression and utility of STAT6 immunohistochemistry for SFTs. 1.Chen S, Zheng Y, Chen L, et al. A broad ligament solitary fibrous tumor with Doege-Potter syndrome. Medicine 2018; 97: e12564.2.Zubor P, Kajo K, Szunyogh N, et al. A solitary fibrous tumor in the broad ligament of the uterus. Pathol Res Pract 2007; 203: 555–60.3.Ronchi A, Cozzolino I, Marino FZ, et al. Extrapleural solitary fibrous tumor: A distinct entity from pleural solitary fibrous tumor. An update on clinical, molecular and diagnostic features. Ann Diagn Pathol 2018; 34: 142–50.

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.

A Case of Non-Islet Cell Tumor Hypoglycemia in Metastatic Solitary Fibrous Tumor

Abstract Introduction: Insulin like growth factor (IGF-2) mediated hypoglycemia secondary to solitary fibrous tumor (SFT), also known as Doege-Potter syndrome is a rare paraneoplastic syndrome. The tumor cells produce large amounts of high molecular weight IGF 2 precursor protein called “big IGF-2” which binds to insulin and IGF receptors in liver, muscle and other peripheral tissues. This causes reduced gluconeogenesis and increased uptake of glucose by the muscle and other tissues leading to hypoglycemia. Big IGF-2 also exerts central negative feedback of growth hormone causing reduction of IGF-I production. Most SFTs are benign and localized (approximately 78-88%). As a result, tumor excision alone would often lead to resolution of the hypoglycemia. We present a case of metastatic SFT with multiple metastasis managed with oral prednisone. Clinical Case: A 44-year-old man with metastatic SFT presented with bilateral humeral fractures. He has known metastatic disease to the brain, lung, liver, bony lytic lesions over a course of eleven years. It has progressed despite multiple chemotherapy and radiation therapies. Prior to admission, he had multiple syncopal episodes associated with fasting hypoglycemia. He reported capillary blood glucose values ranging between 30-50 mg/dl during these episodes which would improve after drinking juice or eating candy. There was no history of diabetes mellitus or use of oral hypoglycemic agents or insulin. On admission, he had a capillary blood glucose value of less than 20 mg/dl, which was confirmed by a serum glucose value of 18 mg/dl on basic metabolic panel. His renal, liver and thyroid function tests were normal. Significant labs include: serum glucose 17 mg/dl, C-peptide <0.10 ng/ml (n: 1-4 ng/ml), serum insulin <1.6 Uu/ml (n: <20 Uu/ml), beta-hydroxybutyrate <0.2 mmol/L (n: <0.3), cortisol 10.8 ug/dl(n: 5-15 ug/dl) glucagon 6 pg/ml(ref 8-57 pg/ml), insulin-like growth factor-1 (IGF 1) 20 ng/ml (n: 52-328), and IGF-2 level 380 ng/ml (267-616 ng/ml), improvement in blood glucose from 46 to 111 mg/dl after 1-gram glucagon administration. The IGF-2/IGF-1 ratio of 19 confirmed our clinical suspicion of non-islet cell tumor hypoglycemia (NICTH). He was started on prednisone 20 mg twice daily with marked improvement in hypoglycemia. Conclusion: NICTH is a rare cause of hypoglycemia and should be considered in the differential while evaluating hypoglycemia in malignancy. For diagnosing NICTH, assays for big IGF-II are not commercially available. However, the IGF-II:IGF-I ratio is considered to be a surrogate marker of big IGFII concentration. The normal ratio is 3 and ratio >10 is diagnostic of NICTH. In cases like ours where tumor resection is not possible, glucocorticoids are most effective in management of hypoglycemia by inhibiting big IGF2 production and stimulating gluconeogenesis.

A Rare Cause of Recurrent Hypoglycemia - The “Big-IGF-II” Factor

Abstract Introduction: Hormone secreting tumors are a rare cause of hypoglycemia. Here we present an interesting case of paraneoplastic phenomenon resulting in a non-islet cell tumor causing hypoglycemia (NICTH) from a solitary fibrous tumor (SFT). Clinical Case: An 83-year-old female with a history of hypertension presented with recurrent episodes of lightheadedness and falls for one month. Review of systems was negative except for insignificant weight loss. Her vital signs and physical examination were unremarkable. Only significant finding on initial blood work was a blood glucose level of 30 mg/dL (n: >70mg/dL). Additional work-up of hypoglycemia revealed a low serum free insulin level 0.6 IU/mL (n:1.5 - 14.9 IU/mL), low proinsulin <4 pmol/L (n: <18.8 pmol/L), low C-peptide level 0.14 ng/mL (n: 0.80-3.85 ng/mL), low serum insulin-like growth factor-I (IGF-I) 33 ng/ml (n:34-246 ng/ml) and elevated IGF-II levels 370 ng/mL (n:47-350 ng/mL). Blood sulfonylurea and insulin antibodies screen were negative, ACTH and morning cortisol levels were within normal limits. A CT scan of chest, abdomen and pelvis for neoplastic workup demonstrated a large heterogeneous mass within the lower lobe of the right lung. A CT-guided biopsy disclosed spindle cell neoplasm, positive for signal transducer and activator of transcription-6 (STAT6) and CD34. Reverse transcription-polymerase chain reaction (RT-PCR) confirmed the NGFI-A binding protein 2 (NAB2) and STAT6 fusion which is specific for solitary fibrous tumor (SFT). She underwent surgical resection of the thoracic mass which resulted in complete resolution of her symptoms. Clinical Lessons: NICTH is a paraneoplastic syndrome associated with various benign and malignant tumors including SFT, hemangiopericytoma and metastatic hepatocellular carcinoma to name a few. Our case is an example of NICTH associated with SFT called Doege-Potter syndrome. The prevalence of hypoglycemia is seen in less than 5% of patients with SFT. It is often secondary to ectopic overexpression of unprocessed precursor ‘big’ IGF-II molecule, which binds to IGF-I receptor, resulting in tumorigenesis. An IGF-II/IGF-1 ratio is a sensitive marker and a value of more than 10 suggests excess production of IGF-II and is likely the cause of NICTH. Physicians should be cognizant of NICTH, especially in non-diabetic, otherwise healthy individuals in whom preliminary workup for hypoglycemia is negative and should look to identify any potential tumor as the likely cause. Surgical treatment is often the only curative option. Frequent meals, dextrose infusion, glucagon and growth hormone are bridging modalities until definitive surgical treatment.

DOEGE-POTTER Syndrome in Malignant Neurofibromatous Tumor: A Case Report

DOEGE-POTTER syndrome is a paraneoplastic syndrome in which hypoglycemia results from tumors producing insulin-like growth factor (IGFII). It is most often the solitary fibrous tumor of the pleura. These are rare and may be discovered incidentally, during non-specific respiratory symptoms or during hypoglycemia. Hypoglycemia occurs in large tumors and disappears after surgery, which is the treatment of choice, allowing permanent cure in the majority of cases.

Dacarbazine and Bevacizumab Improved Paraneoplastic Doege–Potter Syndrome of Malignant Solitary Fibrous Tumor

Solitary fibrous tumors (SFTs) are rare soft-tissue tumors that often occur in body cavities, especially the pleural space. A subset of SFTs is histologically malignant and tends to metastasize; rarely, they may induce paraneoplastic syndromes. Doege–Potter syndrome is paraneoplastic hypoglycemia induced by oversecretion of unprocessed insulin-growth factor-2. While localized SFTs are treated mainly by surgery, the standard therapy for metastatic SFTs is lacking. Here, we present a case with metastatic malignant SFT and Doege–Potter syndrome, which was treated initially by dacarbazine and bevacizumab with a period of good clinical response.

Solitary fibrous tumor of the pleura: A rare mesenchymal tumor presented with hypoglycemia

Solitary fibrous tumor (SFT) is a relatively rare primary tumor of the pleura, originating from the mesenchymal cells. The tumor has a non-specific clinical, laboratory, and radiological features. Patients may be asymptomatic or have non-specific clinical features including chest pain, dyspnea, cough, and paraneoplastic symptoms. Complete surgical resection is indicated in all cases to avoid recurrence of the tumor. We described a 49-yearold woman who presented with unexplained right chest pain and repeated attacks of hypoglycemia, associated with pleural SFT. Keywords: Pleura; thoracic oncology; solitary fibrous tumor; paraneoplastic hypoglycemia; Doege-Potter syndrome

Doege-Potter syndrome: a case report

Doege-Potter syndrome is a rare paraneoplastic syndrome presenting as hypoinsulinemic hypoglycemia due to ectopic secretion of prohormone: insulin-like growth factor-2 (IGF-2) from a solitary fibrous tumor (SFT). It is essentially a non-islet cell tumor hypoglycemia (NICTH) or in more recent terms, an ‘IGF-2oma’. In case of delay in diagnosis, tumor-induced hypoglycemia could cause hypoxic cerebral injury or death. We hereby present a case of 45 years old male who presented with hypoglycemia due to pleural SFT, which is a very rare and unique presentation. Upon biochemical and radiological investigations, Doege-Potter syndrome was diagnosed and the patient was planned for surgical resection of the tumor. This case stresses upon thorough evaluation of patients presenting with non-diabetic hypoglycemia to rule out underlying malignancy.

P22.06 A Large Solitary Fibrous Tumor of the Pleura Causing Paraneoplastic Hypoglycemia

Refractory hypoglycemia (Doege-Potter syndrome) arises in <5 % of solitary fibrous tumors, particularly in large pleural/peritoneal tumors. This syndrome is known to be caused by tumor secretion of large insulin-like growth factor 2 (IGF-2) and surgical resection of the tumor is the treatment of choice. A 78-year-old female presented to our emergency department with dizziness for a week. She did not have any previous history or any medications. Chest computed tomography revealed a 25-cm large tumor occupying the left hemithorax, shifting the mediastinum to the right (Arrows in Figure 1A). She was emergently admitted for concerning cardiovascular collapse. She developed syncope the next morning. Hypoglycemia (29 mg/dL), which was not apparent on admission, was revealed at that time. She responded to intravenous glucose immediately. In a systemic workup, no other lesions outside was documented. A pulmonary perfusion scan demonstrated only 1.8% in the left side. A percutaneous biopsy of the tumor revealed a solitary fibrous tumor. The hypoglycemia appeared to be a paraneoplastic syndrome (Doege-Potter syndrome). We resected the tumor through a clamshell incision (Arrows in Figure 1B). A left pneumonectomy was necessary (Figure 2).View Large Image Figure ViewerDownload Hi-res image Download (PPT) The hypoglycemia resolved immediately after removal of the tumor. The patient was discharged home on the 12th postoperative day. There were no complications. A pathological examination confirmed the diagnosis of solitary fibrous tumor with morphological features of malignancy. She was asymptomatic without recurrence 4 months after surgery. The patient underwent a successful surgical treatment, which maintains recurrence-free survival in mid-term.

Solitary fibrous tumor of the pleura with Doege-Potter syndrome.

Solitary fibrous tumor of pleura (SFTP) is a rare tumor. Complete tumor resection is the curative treatment and the key to preventing recurrence.&#x0D; We present here the case of a 65-year-old female diagnosed with a Doege-Potter syndrome who underwent resection of pleural solitary fibrous tumor.

Síndrome de Doege-Potter: uma apresentação rara de tumor fibroso solitário da pleura

Síndrome de Doege-Potter: uma apresentação rara de tumor fibroso solitário da pleura

P41 Case Report: An Interesting Presentation of Hypoglycaemia

Doege-Potter syndrome (DPS) is a rare paraneoplastic syndrome associated with solitary fibrous tumours and is famously characterised by hypoglycaemia. We present one new case report of DPS which was surgically treated in The Townsville Hospital.

Refractory hypoglycemia associated with giant solitary fibrous tumor of the pleura (Doege–Potter syndrome)

The association of a solitary tumor of the pleura with paraneoplastic refractory hypoglycemia is a very rare clinical condition. An 86-year-old gentleman with an incidentally discovered large mass in the left chest and a long-standing history of refractory hypoglycemia was referred for surgical consultation. Computed tomography and PET (Positron Emission Tomography) scan confirmed the presence of a homogeneous mass leaning on the left hemidiaphragm with no tracer uptake. After tumor board discussion, the mass was radically excised through a left thoracotomy. Histopathology revealed a benign solitary fibrous tumor of the pleura. At 1‑year follow-up the patient was doing well and did not further complain of hypoglycemia-related symptoms. Doege–Potter syndrome is a life-threatening condition associated with severely impaired quality of life. Prompt identification and surgical treatment are mandatory.

Síndrome de Doege Potter

El síndrome de Doege Potter es un síndrome caracterizado por hipoglucemia severa asociada a tumor fibroso de la pleura. Presentamos el caso de una paciente de 67 años con antecedente por biopsia de tumor fibroso de pleura, quien seis meses después de este diagnóstico presenta episodios de alteración del estado de conciencia con desorientación y documentación de hipoglucemia con triada de Whipple presente. Se realiza test de ayuno el cual es positivo para hipoglucemia no hiperinsulinémica y dado sus antecedentes, se hace el diagnóstico de un síndrome de Doege Potter. Se realiza manejo quirúrgico con resección total de masa tumoral con posterior resolución de la hipoglucemia

Clinical Presentation, Natural History, and Therapeutic Approach in Patients with Solitary Fibrous Tumor: A Retrospective Analysis.

Background Solitary fibrous tumor (SFT) is a rare variant of soft tissue sarcoma (STS). Materials and Methods. We reviewed SFT patients (pts) treated at our institution between 12/1990 and 09/2017. Results We identified 94 pts with a median follow-up (mFU) of 4.7 years (range: 0.1–21.53). Primary sites were the chest (33%), abdomen (21.3%), brain (12.8%), and extremities (9.6%); 6.4% of pts presented with synchronous metastasis. Median overall survival (mOS) from the first diagnosis was 56.0 months (m) (0.3–258.3). Doege–Potter syndrome was seen in 2.1% of pts. Primary resection was performed in 86 pts (91.5%). Median progression-free survival was 34.1 m (1.0–157.1), and 43% of pts stayed SFT-free during FU. Local recurrence occurred in 26.7% after a mFU of 35.5 m (1.0–153.8), associated with an OS of 45.1 m (4.7–118.2). Metachronous metastasis occurred in 30.2% after a mFU of 36.0 m (0.1–157.1). OS in metastatic pts was 19.0 m (0.3–149.0). Systemic therapy was given to 26 pts (27.7%) with inoperable/metastatic disease. The most common (57.7%) upfront therapy was doxorubicin, achieving responses in 13.3% of pts with a PFS of 4.8 m (0.4–23.8). In second line, pts were treated with ifosfamide or pazopanib, the latter achieving the highest response rates. Third-line treatment was heterogeneous. Conclusion SFT is an orphan malignancy with a highly variable clinical course and a considerable risk of local failure and metachronous metastasis. Surgery is the only curative option; palliative systemic therapy is used in inoperable/metastatic cases but achieves low response rates. The highest response rates are seen with pazopanib in second/third line.

Metastatic solitary fibrous tumor of the pancreas in a patient with Doege-Potter syndrome.

A 46-year-old man was admitted to our hospital with a 6-month history of recurrent hypoglycemia with an unknown cause. Four months back, he was diagnosed with a complex ulcer in the stomach and duodenum in a local hospital.

Giant pleural fibrous tumor: a case report

Pleural fibrous tumor (PFT) is an infrequent and indolent pleural tumor, and most patients remain asymptomatic for years. The diagnosis is usually incidental and in large-size cases, representing a major surgical challenge. They may be associated to paraneoplastic syndromes such as refractory hypoglycemia or Doege-Potter syndrome. The present case is a 50-year-old female who came with dyspnea, chronic cough and constitutional syndrome. The CT scan showed a large intrathoracic mass of 20×18×17 cm 3 which compressed the right lower and middle lobes (RLL and RML). At the time of the operation, the approach was right lateral thoracotomy extended to the chondrocostal region. During the resection, the tumor was accidentally opened and at that time the patient showed hypoglycemic levels. It is not often a long size PFT with hypoglycemia at the moment of operation without previous diagnosis. PFT is usually asymptomatic and is diagnosed incidentally in half of the cases. Tumor resection is the treatment of choice, which also leads to the cure of paraneoplastic syndrome. For tumors larger than 5 cm, thoracotomy is preferred, and both the tumor and its implant pedicle must be resected with safety margins.

Rehabilitation nursing of a patient with pleural giant solitary fibroma with Doege-Potter syndrome

Objective To summarize the clinical rehabilitation nursing practice of a patient with pleural giant solitary fibroma with Dege-Port syndrome. Methods A preoperative and postoperative rehabilitation nursing of a patient with low pulmonary function pleural giant solitary fibroma and non-islet cell hypoglycemia was analyzed and summarized. Results After psychological, hypoglycemia, and pulmonary rehabilitation, the patient was tolerant to surgery and recovered well after surgery. He was discharged from hospital on the 13th day after surgery. Conclusions For patients with difficult diagnosis, long-term pleural giant solitary fibroma with hypoglycemia and low lung function, psychological nursing, hypoglycemia symptomatic treatment, pulmonary rehabilitation to improve lung function, close observation and prevention of serious complications in patients It plays a vital role in the course of the disease and rehabilitation. Key words: Solitary fibrous tumor; Rehabilitation nursing; Doege-Potter syndrome

Solitary fibrous tumor: A Brazilian cohort analysis.

11068 Background: Solitary fibrous tumor (SFT) is a rare mesenchymal tumor that account for less than 2% of all soft tissue sarcomas. SFT has been identified in multiple anatomic locations and can arise anywhere in the body. Surgical management is the mainstay of treatment for localized disease. However, about 20% will develop locoregional recurrences or distant metastasis with a role for systemic treatment. Methods: A retrospective analysis was carried out in a large cancer center in Brazil. Our primary objective was to evaluate clinical and treatment aspects of metastatic/ locally advanced (Mtx/LA) SFT cohort and secondary to describe clinical characteristics of entire population diagnosed with SFT. Descriptive statistics was used for main results. Survival curves were estimated using Kaplan-Meier. Data were retrieved from electronic patient medical records. Results: From April, 1971 to October, 2017, 82 patients with SFT were treated. Median follow-up was 45.5 months. 67 (81.7%) were alive on the cut-off date. Median age at diagnosis was 51 (14-78). 40.2% men. Most common primary sites (PS) were pleura (19.8%), central nervous system (CNS - 11%) and pelvis (11%). 18 (21.9%) underwent chemotherapy for Mtx/LA disease. In this subgroup, 61.1% were men; PS retroperitoneal (22.2%), extremities (16.7%), CNS (16.7%). 66.7% had pulmonary, 44.4% hepatic, 27.8% bone metastasis and one (5.5%) local recurrence. All patients had at least one adverse prognostic factor (tumor size ≥ 10cm, positive margins, necrosis, ≥ 4/10 mitosis). One (5,5%) had Doege-Potter syndrome. 7 (38.9%) did one, 5 (27.8%) two and 6 (33.3%) ≥ 3 lines of treatment. First line was temozolomide/bevacizumab (TMZ/Bev) in 55.6%, followed by chemotherapy (Ch) in 27.8% and tyrosine kinase inhibitors (TKI) in 16.7%. Median progression-free survival was 3.5 months (95% IC: 0.0-7.4) and overall survival 27.3 months (95% IC: 18.7-36.0). Response rate using RECIST criteria was 12.5% for TMZ/Bev and 62.5% had stable disease. TKI and Ch had no response. Conclusions: SFT is rare and with heterogeneous clinical presentation. In our analysis, patients received a wide range of therapy, reflecting the lack of well-established systemic treatment option. TMZ/Bev showed consistent activity in Mtx/LA scenario.