scholarly journals Oncogenic osteomalacia secondary to glomus tumor

2021 ◽  
Vol 2021 ◽  
Author(s):  
Rishi Raj ◽  
Samaneh Hasanzadeh ◽  
Mitra Dashtizadeh ◽  
Mohammadreza Kalantarhormozi ◽  
Katayoun Vahdat ◽  
...  
Keyword(s):  
Surgical Resection ◽  
Glomus Tumor ◽  
Clinical Manifestations ◽  
List Type ◽  
Oncogenic Osteomalacia ◽  
Mesenchymal Tumors ◽  
Elevated Alkaline Phosphatase ◽  
Sinonasal Mass ◽  
History Of ◽  
Octreotide Scan

Summary Oncogenic osteomalacia secondary to glomus tumor is extremely rare. Localization of causative tumors is critical as surgical resection can lead to a complete biochemical and clinical cure. We present a case of oncogenic osteomalacia treated with resection of glomus tumor. A 39-year-old woman with a history of chronic sinusitis presented with chronic body ache and muscle weakness. Biochemical evaluation revealed elevated alkaline phosphatase hypophosphatemia, increased urinary phosphate excretion, low calcitriol, and FGF23 was unsuppressed suggestive of oncogenic osteomalacia. Diagnostic studies showed increase uptake in multiple bones. Localization with MRI of paranasal sinuses revealed a sinonasal mass with concurrent uptake in the same area on the octreotide scan. Surgical resection of the sinonasal mass was consistent with the glomus tumor. The patient improved both clinically and biochemically postoperatively. Along with the case of oncogenic osteomalacia secondary to a glomus tumor, we have also discussed in detail the recent development in the diagnosis and management of oncogenic osteomalacia. Learning points Tumor-induced osteomalacia is a rare cause of osteomalacia caused by the secretion of FGF23 from mesenchymal tumors. Mesenchymal tumors causing TIO are often difficult to localize and treat. Resection of the tumor can result in complete resolution of biochemical and clinical manifestations in a very short span of time. Glomus tumor can lead to tumor induced osteomalacia and should be surgically treated.

Oncogenic osteomalacia caused by a phosphaturic mesenchymal tumor of the thoracic spine

2009 ◽  
Vol 10 (4) ◽  
pp. 329-333 ◽  
Author(s):  
Elena Pirola ◽  
Francesco Vergani ◽  
Paolo Casiraghi ◽  
Eugenio Biagio Leone ◽  
Paolo Guerra ◽  
...  
Keyword(s):  
Thoracic Spine ◽  
Paraneoplastic Syndrome ◽  
Tumor Resection ◽  
Mesenchymal Tumor ◽  
Phosphaturic Mesenchymal Tumor ◽  
Oncogenic Osteomalacia ◽  
Mesenchymal Tumors ◽  
History Of ◽  
Complete Tumor

Phosphaturic mesenchymal tumors that cause the paraneoplastic syndrome known as oncogenic osteomalacia are rare. The authors report on the case of a 57-year-old man with a history of osteomalacia and in whom was diagnosed a thoracic spine tumor at the T-4 level. Complete tumor resection was accomplished. The histological diagnosis was phosphaturic mesenchymal tumor (mixed connective tissue variant). After lesion removal, the paraneoplastic syndrome resolved. At the 24-month follow-up, no recurrence of the disease was observed. The clinical presentation, surgical technique, and follow-up in this case were reviewed in detail.

scholarly journals A rare case of ectopic ACTH syndrome caused by primary renal neuroendocrine tumor

2021 ◽  
Vol 2021 ◽  
Author(s):  
Paweena Chunharojrith ◽  
Kanapon Pradniwat ◽  
Tanawan Kongmalai
Keyword(s):  
Neuroendocrine Tumor ◽  
Multimodality Imaging ◽  
Left Kidney ◽  
Clinical Manifestations ◽  
Biological Behavior ◽  
Ectopic Acth Syndrome ◽  
List Type ◽  
Lower Pole ◽  
Ectopic Acth ◽  
Octreotide Scan

Summary Ectopic adrenocorticotropic hormone (ACTH) secretion is responsible for 5–15% of Cushing’s syndrome (CS). Neuroendocrine tumor (NET) is a common cause of ectopic ACTH syndrome (EAS). However, primary renal NET is exceedingly rare. Fewer than 100 cases have been reported and only a few cases presented with CS. Because of its rarity and lack of long-term follow-up data, clinical manifestations, biological behavior and prognosis are not well understood. Here, we report the case of a 51-year-old man who presented with clinical and laboratory findings compatible with EAS. CT scan revealed a lesion of uncertain nature at the lower pole of the left kidney. Octreotide scan found a filling defect at the lower pole of left kidney. It was difficult to determine if this finding was the true etiology or an incidental finding. Unfortunately, the patient’s clinical status rapidly deteriorated with limited medical treatment. The patient underwent left nephrectomy and left adrenalectomy. Histopathological examination confirmed NET with oncocytic features. Immunohistochemistry staining was positive for ACTH. The patient’s condition gradually improved. Additionally, glucocorticoid replacement was required only 6 months during a gradual recovery of hypothalamic pituitary adrenal axis achieved approximately three years after tumor removal. Although extremely rare, primary renal NET should be considered as a cause of EAS particularly in a patient with rapid clinical deterioration. Thorough investigation, early diagnosis and careful management are crucial to reduce morbidity and mortality. Learning points Primary renal NET is an extremely rare cause of ectopic ACTH syndrome. Ectopic ACTH syndrome has a rapid onset with severe clinical manifestations. In this case, the patient’s condition deteriorated rapidly, resulting from severe hypercortisolism. Resection of the tumor is the most effective treatment. Localization of ectopic ACTH-secreting tumors is very challenging. Multimodality imaging including CT, MRI, octreotide scan, and positron emission tomography plays a crucial role in identifying the tumors. However, each imaging modality has limitations.

scholarly journals Glomus Tumor: An Unusual Cause of Hypophosphatemic Osteomalacia

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A195-A196
Author(s):  
Rishi Raj ◽  
Samaneh Hasanzadeh ◽  
Mitra Dashtizadeh ◽  
Mohammadreza Kalantarhormozi ◽  
Katayoun Vahdat ◽  
...  
Keyword(s):  
Serum Creatinine ◽  
Glomus Tumor ◽  
Clinical Symptoms ◽  
Clinical Manifestations ◽  
Serum Phosphate ◽  
Whole Body ◽  
Positive Staining ◽  
Urine Creatinine ◽  
Sinonasal Mass ◽  
Hypophosphatemic Osteomalacia

Abstract Background: Tumor-induced osteomalacia (TIO) is a rare condition resulting in hypophosphatemic osteomalacia. We present a rare case of TIO secondary to glomus tumor. Clinical Case: A 39-year-old woman with history of chronic sinusitis presented with progressively worsening generalized body pain and muscle weakness of eight months duration. Examination showed decreased muscle strength in bilateral upper and lower extremities and congenital cleft palate. Laboratory work up revealed elevated alkaline phosphatase 603 U/L (44–147 U/L), low serum phosphorus 1.5 mg/dL (3.5–5.0 mg/dL), normal serum calcium 8.9 mg/dL (8.3–10.4 mg/dL), normal 25-hydroxyvitamin D 32 ng/dL (30–100 ng/dL), elevated 1,25-dihydroxyvitamin D 62 g/mL (20–45 pg/mL), elevated intact PTH level 99.01 pg/mL (8–74 pg/mL), high normal 24 hour urinary phosphate levels 1100 mg/dl, and elevated FGF23 level 128 RU/mL (<108 RU/mL). Fractional excretion of filtered phosphate (FEPO4) [FEPO4 = (Urine phosphate / Serum phosphate) / (Urine creatinine / Serum creatinine) * 100] was 107.54 % (normal range <20%). Tubular reabsorption of phosphate (TRP) [Percentage TRP = 100 * {1- (Urine phosphate / Serum phosphate) / (Urine creatinine / Serum creatinine)}] was -7.54 %. Tubular maximum reabsorption of phosphate to glomerular filtration rate (TmP/GFR) calculated using formula [TmP/GFR = TRP x serum phosphate] was -0.1131 mg /dL (2.8–4.2 mg/dL). Bone densitometry revealed normal bone density and parathyroid scintigraphy was negative for adenoma. Clinical symptoms along with biochemical evidence of phosphate wasting confirmed by low TRP and an unsuppressed FGF23 levels suggested oncogenic osteomalacia as underlying etiology. Whole body bone scan demonstrated increased radiotracer uptake in the elbows, spine, sacroiliac joints, knees, ankles and multiple ribs suggestive of inflammatory process. MRI of paranasal sinuses revealed large soft tissue mass occupying right frontal, ethmoid, maxillary and sphenoid sinuses suggestive of sinonasal polyposis. 99mTc-Octreotide scan confirmed increased uptake in the nasopharynx. The sinonasal mass was resected and pathology revealed the mass to be a glomus tumor confirmed on immune histochemical studies with positive staining for vimentin and SMA and Ki67 of 5–10 %. Postoperatively, phosphorus levels normalized to 3 mg/dl on day 7. Conclusion: Glomus tumor as cause of TIO is extremely rare however localization and surgical resection of tumor can result in complete resolution of biochemical and clinical manifestations. References: 1. Minisola, S., et al., Tumour-induced osteomalacia. Nat Rev Dis Primers, 2017. 3: p. 17044.

scholarly journals Status epilepticus and diabetes ketoacidosis: uncommon clinical presentations of acromegaly

2021 ◽  
Vol 2021 ◽  
Author(s):  
Nyasatu G Chamba ◽  
Ahlam A Amour ◽  
Abid M Sadiq ◽  
Tecla R Lyamuya ◽  
Emmanuel V Assey ◽  
...  
Keyword(s):  
Status Epilepticus ◽  
Elevated Serum ◽  
Clinical Manifestations ◽  
Mass Effect ◽  
Prolactin Secretion ◽  
List Type ◽  
Resource Setting ◽  
Clinical Presentations ◽  
History Of ◽  
New Onset

Summary Acromegaly is a rare disease caused by hypersecretion of the growth hormone (GH). Most cases are caused by either pituitary microadenoma or macroadenoma. The GH producing tumors present with clinical manifestations of acromegaly due to excessive GH secretion or symptoms resulting from mass effects of the enlarging tumor. The physical changes are usually slow and, therefore, recognition of the disease is delayed. These adenomas are never malignant but can have significant morbidity and mortality. A subgroup of patients with acromegaly present with severe hyperglycemia resulting in diabetic ketoacidosis (DKA) which requires insulin. Rarely are pituitary tumors responsible for generalized convulsions except when they are too large. We hereby present two cases, the first is that of a 26-year-old female who presented with new onset status epilepticus, DKA with a 1-year history of diabetes mellitus (DM). On examination, she had clinical features of acromegaly. The second case is that of a 34-year-old female who presented with new onset status epilepticus, hyperglycemia with a history of recently diagnosed DM, and features of gigantism. In both cases, their diagnosis was confirmed by elevated serum GH and later by elevated insulin-like growth factor type 1 levels, and CT of the head demonstrating large pituitary macroadenoma. The importance of clinical history and examination, as well as investigations is vital in the recognition of acromegaly. The prognosis of acromegalic patients depends on early clinical recognition and tumor size reduction by either medical or surgical therapy. Learning points Conditions such as status epilepticus and DKA may be clinical presentations in patients presenting with acromegaly. Seizures are rare in people with pituitary adenoma and typically occur when the tumor invades the suprasellar area due to mass effect on the brain. This article shows how best we were able to manage the acromegaly complications in a low resource setting. Hyperprolactinemia in acromegaly may be due to disruption of the normal dopaminergic inhibition of prolactin secretion due to mass effect of the macroadenoma, and around 25% of GH-secreting adenomas co-secrete prolactin.

scholarly journals Hypoglycemia after bariatric surgery: importance of exhaustive hormonal study

2021 ◽  
Vol 2021 ◽  
Author(s):  
Alejandra Perez-Montes de Oca ◽  
Silvia Pellitero ◽  
Manel Puig-Domingo
Keyword(s):  
Bariatric Surgery ◽  
Gh Deficiency ◽  
Clinical Manifestations ◽  
Clinical Problem ◽  
Medical Problem ◽  
Dumping Syndrome ◽  
Diabetic Patients ◽  
List Type ◽  
Laboratory Findings ◽  
History Of

Summary Hypoglycemia is an uncommon clinical problem in non-diabetic patients or patients not being treated for diabetes mellitus. It is a rare, but well-established complication of bariatric surgery and, in some cases, it can be the only symptom of another medical problem. A 50-year-old woman with a history of partially recovered hypopituitarism after transsphenoidal surgery for a non-functioning pituitary macroadenoma complained about symptomatic hypoglycemia after sleeve gastrectomy surgery. Our initial studies failed to determine the cause for these episodes and treatment with acarbose (suspecting a dumping syndrome) was not helpful. Finally, laboratory findings revealed growth hormone (GH) deficiency. The patient received treatment with GH, with the resolution of symptoms after 3 months of treatment. Our case suggests that all causes of hypoglycemia should be considered and studied after bariatric surgery. An improvement in insulin-resistance following bariatric surgery can trigger clinical manifestations of GH deficiency. Learning points Postprandial hypoglycemia after bariatric surgery is usually due to dumping syndrome. Even after bariatric surgery, all causes of hypoglycemia should be considered and studied. After significant weight loss, insulin sensitivity is usually restored and can trigger clinical manifestations of GH deficiency. Hypoglycemia is a rare symptom of GH deficiency.

Etiology and clinical features of epididymo-orchitis: A single-center study in Tehran, Iran

2020 ◽  
Vol 20 ◽  
Author(s):  
Sara Abolghasemi ◽  
Mohammad Alizadeh ◽  
Ali Hashemi ◽  
Shabnam Tehrani
Keyword(s):  
Chlamydia Trachomatis ◽  
Clinical Symptoms ◽  
Urine Culture ◽  
Clinical Manifestations ◽  
Urinary Frequency ◽  
Serological Tests ◽  
Duration Of Symptoms ◽  
Two Samples ◽  
History Of ◽  
Tract Infections

Introduction: Epididymo-orchitis is a common urological disease among men. Little is known about the clinical and epidemiological aspects of the disease in Iran. Thus, the present study was aimed to investigate the etiology, clinical sequelae and risk factors of patients with epididymo-orchitis in Tehran, Iran. Materials and Methods: Patients presenting with epididymo-orchitis were prospectively analyzed in order to study the etiology and pattern of the disease. Bacteriological, molecular and serological tests were undertaken to look for Chlamydia trachomatis, Neisseria gonorrhoeae, Brucella spp., Mycoplasma spp, and other bacteria. Results: Fifty patients with epididymo-orchitis were evaluated according to their clinical symptoms, duration of symptoms, physical examination, and laboratory studies. The mean age of the patients was 53 years. Fever, dysuria, pain in the flanks, urinary frequency and discharges occurred in 58.0%, 50.0%, 50.0%, 28.0% and 6.0%, respectively. Bacterial pathogen was identified in 26% (13/50) of patients by urine culture. Escherichia coli was the etiological agent in 11/13 patients (84.6%). Two out of 50 patients (4.0%) were also positive for Chlamydia trachomatis. Two samples were serologically positive for Brucella spp. High Mean age, fever, urinary frequency, history of the underlying disease and history of urinary tract infections were found to have a significant association with the positive bacteriologic urine culture (P<0.05). Conclusions: The most common clinical manifestations were fever, dysuria, and abdominal pain. E. coli and C. trachomatis were the major causative agents. Use of a set of diagnostic approaches including clinical symptoms, urine culture and more precise techniques such as PCR should be taken into consideration for the definitive diagnosis.

The physiopathology of spontaneous hemorrhagic stroke: a systematic review

2021 ◽  
Vol 0 (0) ◽  
Author(s):  
Alcivan Batista de Morais Filho ◽  
Thiago Luis de Holanda Rego ◽  
Letícia de Lima Mendonça ◽  
Sulyanne Saraiva de Almeida ◽  
Mariana Lima da Nóbrega ◽  
...  
Keyword(s):  
Risk Factors ◽  
Systematic Review ◽  
Ischemic Stroke ◽  
Subarachnoid Hemorrhage ◽  
Natural History ◽  
Hemorrhagic Stroke ◽  
Recent Literature ◽  
Clinical Manifestations ◽  
The Road ◽  
History Of

Abstract Hemorrhagic stroke (HS) is a major cause of death and disability worldwide, despite being less common, it presents more aggressively and leads to more severe sequelae than ischemic stroke. There are two types of HS: Intracerebral Hemorrhage (ICH) and Subarachnoid Hemorrhage (SAH), differing not only in the site of bleeding, but also in the mechanisms responsible for acute and subacute symptoms. This is a systematic review of databases in search of works of the last five years relating to the comprehension of both kinds of HS. Sixty two articles composed the direct findings of the recent literature and were further characterized to construct the pathophysiology in the order of events. The road to the understanding of the spontaneous HS pathophysiology is far from complete. Our findings show specific and individual results relating to the natural history of the disease of ICH and SAH, presenting common and different risk factors, distinct and similar clinical manifestations at onset or later days to weeks, and possible complications for both.

scholarly journals Undifferentiated pleomorphic sarcoma in oropharyngeal mucosa of patients with multiple basal cell carcinomas

Rare Tumors ◽  
2021 ◽  
Vol 13 ◽  
pp. 203636132110264
Author(s):  
Andrea Dekanić ◽  
Marko Velepič ◽  
Margita Belušić Gobić ◽  
Ita Hadžisejdić ◽  
Nives Jonjić
Keyword(s):  
Differential Diagnosis ◽  
Basal Cell ◽  
Polypoid Lesion ◽  
Older Patient ◽  
Mesenchymal Tumors ◽  
Undifferentiated Pleomorphic Sarcoma ◽  
Pleomorphic Sarcoma ◽  
Basal Cell Carcinomas ◽  
History Of ◽  
Multiple Basal Cell Carcinomas

Malignant mesenchymal tumors of oropharyngeal mucosa are rare. Those with fibroblastic and histiocytic differentiation in the skin are called atypical fibroxanthoma (AFX) and in the soft tissue undifferentiated pleomorphic sarcoma (UPS). Here we present a case of an older patient with a history of multiple basal cell carcinomas and recently with a rapidly growing polypoid lesion in the mucosa of posterior oropharyngeal wall with AFX/UPS morphology. The differential diagnosis, histological pitfalls of this poorly characterized mesenchymal lesions, and the challenges associated with treatment are discussed.

Acute exudative paraneoplastic polymorphous vitelliform maculopathy in a patient with thymoma, myasthenia gravis, and polymyositis

2021 ◽  
pp. 112067212199404
Author(s):  
He Yu ◽  
Xinyu Ma ◽  
Nianting Tong ◽  
Zhanyu Zhou ◽  
Yu Zhang
Keyword(s):  
Myasthenia Gravis ◽  
Medical Center ◽  
Postoperative Radiotherapy ◽  
Clinical Manifestations ◽  
Subretinal Fluid ◽  
Metastatic Tumor ◽  
The Body ◽  
Pleural Thickening ◽  
History Of ◽  
Clinically Significant

Importance: This is the first reported case of acute exudative paraneoplastic polymorphous vitelliform maculopathy (AEPPVM) in a patient with thymoma, accompanied by myasthenia gravis (MG) and polymyositis. Objective: To examine the pathogenesis of ocular disease in a patient with yolk-like fundus lesions and thymoma, MG, and polymyositis throughout the body based on clinical manifestations, diagnosis, differential diagnosis, and genetic testing to determine the appropriate treatment course. Design, setting, and participants: We describe a 63-year-old woman who presented to our tertiary medical center with a 3-month history of reduced visual acuity in both eyes. Concurrent fundoscopy revealed a 2.0 × 1.7-mm, unifocal, yellow, round vitelliform lesion in the macular region, surrounded by multifocal, shallow, yellow-white pockets of subretinal fluid. The patient’s medical history included thymoma with thymectomy treatment, combined with pericardiectomy and postoperative radiotherapy (20 years prior), followed by a diagnosis of MG with suspect thymic association (15 years prior). Three years prior, the patient had been diagnosed with polymyositis related to paraneoplastic syndrome; 1 year prior, she had been examined for pleural thickening due to suspected metastatic tumor. Results: On her most recent follow-up visit at 3 months after initial diagnosis, the patient was stable with no clinically significant progression in ocular or systemic conditions.

Sign in / Sign up

Export Citation Format

Share Document