Successful Multimodal Treatment of an IGF2-Producing Solitary Fibrous Tumor With Acromegaloid Changes and Hypoglycemia

Abstract Doege–Potter syndrome with acromegaloid facial changes is extremely rare. Uncooked cornstarch along with glucocorticoids have been used as supportive care in patients with non–islet cell tumor hypoglycemia (NICTH). Preoperative embolization of hepatic solitary fibrous tumors (SFT) with NICTH has yielded unsatisfactory results. Herein we present the case of a 61-year-old man with a 3-month history of severe frequent hypoglycemic episodes and acromegaloid facial changes. During a spontaneous hypoglycemia (26 mg/dL), laboratory values showed a hypoinsulinemic pattern with low levels of GH, IGFPB3, and an IGF2/IGF1 ratio of 8.5:1. Cross-sectional imaging revealed a large (16 × 13 × 11 cm) hepatic tumor, and cytology was consistent with SFT. A preoperative right portal embolization was performed in an effort to induce normal remnant liver hypertrophy to allow for safe tumor resection. After the procedure, uncooked starch treatment followed by prednisone was started, achieving complete remission of hypoglycemic episodes in the preoperative setting. He subsequently underwent partial hepatectomy. The histologic diagnosis was compatible with a potentially malignant SFT. The patient had an excellent outcome with complete remission of hypoglycemia, improvement of facial acromegaloid changes, and no further evidence of disease. To our knowledge, this is the first case of a patient with Doege–Potter syndrome with acromegaloid facial changes induced by a potentially malignant liver SFT, treated successfully with a multimodal approach consisting of uncooked cornstarch, low-dose prednisone, preoperative embolization, and complete surgical resection. The use of cornstarch and low-dose glucocorticoids may be an adequate treatment in advance of undergoing surgery.

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Preoperative Embolization of Brain Tumor with Pial Artery or Dural Branch of Internal Carotid Artery as Feeding Artery

Interventional Neuroradiology ◽

10.1177/15910199060120s145 ◽

2006 ◽

Vol 12 (1_suppl) ◽

pp. 246-251 ◽

Cited By ~ 3

Author(s):

M Hirohata ◽

T Abe ◽

N Fujimura ◽

Y Takeuchi ◽

M Shigemori

Keyword(s):

Brain Tumor ◽

Carotid Artery ◽

Blood Loss ◽

Internal Carotid Artery ◽

Internal Carotid ◽

Tumor Resection ◽

Preoperative Embolization ◽

Digital Subtraction ◽

Feeding Artery ◽

Pial Artery

This study evaluated the clinical usefulness of preoperative embolization of the pial artery or dural branch of the internal carotid artery (ICA) for brain tumor. Subjects comprised 17 patients with large hypervascular brain tumors who underwent preoperative selective embolization. Micro-catheters (2- or 1.7-F) and shapeable hydrophilic micro-guide wires were used for pial artery (branching from the posterior or anterior cerebral artery) or ICA dural branch embolization. Embolization was performed under digital subtraction fluoroscopy with Polyvinyl alcohol (PVA) particles (150–250 mm) and/or liquid coil. Tumor resection was performed immediately or the day after embolization. Digital subtraction angiography (DSA) before and after endovascular devascularization, blood loss during tumor resection, and clinical outcome were evaluated. All endovascular procedures were technically successful. Post-embolization DSA revealed either a disappearance or a marked decrease of the tumor stain in all cases. Control of intraoperative bleeding was easily accomplished, and intraoperative blood loss was low. Preoperative particle embolization of the feeding artery from the cortical artery or ICA dural branch is safe and effective as adjuvant therapy before tumor resection.

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Sclerostin antibody stimulates periodontal regeneration in large alveolar bone defects

Scientific Reports ◽

10.1038/s41598-020-73026-y ◽

2020 ◽

Vol 10 (1) ◽

Cited By ~ 1

Author(s):

Yao Yao ◽

Frederic Kauffmann ◽

Shogo Maekawa ◽

Lea V. Sarment ◽

James V. Sugai ◽

...

Keyword(s):

Targeted Drug Delivery ◽

Low Dose ◽

Alveolar Bone ◽

Tumor Resection ◽

Periodontal Regeneration ◽

Neutralizing Antibody ◽

Bone Defects ◽

Dose Delivery ◽

Osseous Defects ◽

Sclerostin Antibody

Abstract Destruction of the alveolar bone in the jaws can occur due to periodontitis, trauma or following tumor resection. Common reconstructive therapy can include the use of bone grafts with limited predictability and efficacy. Romosozumab, approved by the FDA in 2019, is a humanized sclerostin-neutralizing antibody (Scl-Ab) indicated in postmenopausal women with osteoporosis at high risk for fracture. Preclinical models show that Scl-Ab administration preserves bone volume during periodontal disease, repairs bone defects surrounding dental implants, and reverses alveolar bone loss following extraction socket remodeling. To date, there are no studies evaluating Scl-Ab to repair osseous defects around teeth or to identify the efficacy of locally-delivered Scl-Ab for targeted drug delivery. In this investigation, the use of systemically-delivered versus low dose locally-delivered Scl-Ab via poly(lactic-co-glycolic) acid (PLGA) microspheres (MSs) was compared at experimentally-created alveolar bone defects in rats. Systemic Scl-Ab administration improved bone regeneration and tended to increase cementogenesis measured by histology and microcomputed tomography, while Scl-Ab delivered by MSs did not result in enhancements in bone or cemental repair compared to MSs alone or control. In conclusion, systemic administration of Scl-Ab promotes bone and cemental regeneration while local, low dose delivery did not heal periodontal osseous defects in this study.

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Carbidopa for Afferent Baroreflex Failure in Familial Dysautonomia

Hypertension ◽

10.1161/hypertensionaha.120.15267 ◽

2020 ◽

Vol 76 (3) ◽

pp. 724-731

Author(s):

Lucy Norcliffe-Kaufmann ◽

Jose-Alberto Palma ◽

Jose Martinez ◽

Horacio Kaufmann

Keyword(s):

Blood Pressure ◽

Blood Pressure Variability ◽

Low Dose ◽

Familial Dysautonomia ◽

Reduce Blood Pressure ◽

Catecholamine Release ◽

High Dose ◽

Double Blind ◽

Adequate Treatment ◽

Baroreflex Failure

Afferent lesions of the arterial baroreflex occur in familial dysautonomia. This leads to excessive blood pressure variability with falls and frequent surges that damage the organs. These hypertensive surges are the result of excess peripheral catecholamine release and have no adequate treatment. Carbidopa is a selective DOPA-decarboxylase inhibitor that suppresses catecholamines production outside the brain. To learn whether carbidopa can inhibit catecholamine-induced hypertensive surges in patients with severe afferent baroreflex failure, we conducted a double-blind randomized crossover trial in which patients with familial dysautonomia received high dose carbidopa (600 mg/day), low-dose carbidopa (300 mg/day), or matching placebo in 3 4-week treatment periods. Among the 22 patients enrolled (13 females/8 males), the median age was 26 (range, 12–59 years). At enrollment, patients had hypertensive peaks to 164/116 (range, 144/92 to 213/150 mm Hg). Twenty-four hour urinary norepinephrine excretion, a marker of peripheral catecholamine release, was significantly suppressed on both high dose and low dose carbidopa, compared with placebo ( P =0.0075). The 2 co-primary end points of the trial were met. The SD of systolic BP variability was reduced at both carbidopa doses (low dose: 17±4; high dose: 18±5 mm Hg) compared with placebo (23±7 mm Hg; P =0.0013), and there was a significant reduction in the systolic BP peaks on active treatment ( P =0.0015). High- and low-dose carbidopa were similarly effective and well tolerated. This study provides class Ib evidence that carbidopa can reduce blood pressure variability in patients with congenital afferent baroreflex failure. Similar beneficial effects are observed in patients with acquired baroreflex lesions.

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Primary cutaneous follicle centre lymphoma, spindle cell type, presenting with multicentre figurated erythema and complete remission after intralesional injections of ultra‐low‐dose Interferon alpha‐2a

Journal of the European Academy of Dermatology and Venereology ◽

10.1111/jdv.16942 ◽

2020 ◽

Author(s):

L. Vlahova ◽

W. Kempf ◽

M.P. Schön ◽

C. Mitteldorf

Keyword(s):

Complete Remission ◽

Interferon Alpha ◽

Low Dose ◽

Spindle Cell ◽

Cell Type ◽

Intralesional Injections ◽

Follicle Centre Lymphoma ◽

Spindle Cell Type

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Low-dose deoxycoformycin in the treatment of hairy cell leukemia

Blood ◽

10.1182/blood.v68.5.1119.1119 ◽

1986 ◽

Vol 68 (5) ◽

pp. 1119-1122 ◽

Cited By ~ 75

Author(s):

EH Kraut ◽

BA Bouroncle ◽

MR Grever

Keyword(s):

Bone Marrow ◽

Complete Remission ◽

Hairy Cell Leukemia ◽

Low Dose ◽

Intravenous Bolus ◽

Hairy Cell ◽

Cell Leukemia ◽

Normal Peripheral Blood ◽

Hairy Cells ◽

Reversible Reduction

Abstract Ten patients with progressive hairy cell leukemia were treated with 2′deoxycoformycin (dCF) by intravenous bolus (4 mg/m2) given every other week. All ten patients are evaluable for response and nine of the ten patients have achieved a complete remission. In addition to clearing of hairy cells from the bone marrow, eight patients had resolution of their monocytopenia. Seven of the nine patients remain in unmaintained remission with a median duration of 6.2 months. Two patients have had relapse in the bone marrow alone and continue to have normal peripheral blood counts. They are being followed without treatment. Toxicity was minimal at this low dose with one patient having a mild reversible reduction in creatinine clearance. Four other patients had reversible neutropenia. There were no significant infections associated with treatment. Low-dose deoxycoformycin administered intravenously every other week represents an extremely effective treatment for hairy cell leukemia.

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The largest reported intrathoracic lipoma: a case report and current perspectives review

Journal of Cardiothoracic Surgery ◽

10.1186/s13019-019-1030-8 ◽

2019 ◽

Vol 14 (1) ◽

Author(s):

Mohammed Aldahmashi ◽

Abdalmotaleb Elmadawy ◽

Mahmoud Mahdy ◽

Mohamed Alaa

Keyword(s):

Single Case ◽

Tumor Resection ◽

Pathological Examination ◽

Complete Excision ◽

Case Presentation ◽

Medical Checkup ◽

Excellent Outcome ◽

The Right ◽

Intrathoracic Mass

Abstract Background The huge size intrathoracic lipomas are very rare. Few cases have been reported worldwide. To our knowledge, this presented case is one of the few cases reported. Here we report a single case as very huge intrathoracic lipoma compressing the right lung and displacing the diaphragm and liver downward. It has been managed by right posterolateral thoracotomy and complete excision, with excellent outcome. Case presentation A 32-year-old male presented with a symptomatic right intrathoracic mass, which was confirmed to be a lipomatous tumor using computed tomography. A penduculated tumor originating from the mediastinal pleura was resected through the conventional right posterior thoracotomy. Pathological examination indicated a diagnosis of fibrolipoma. Conclusion The tumor was symptomatic and relatively huge when detected during a medical checkup. This enabled the successful tumor resection via conventional thoracotomy approach. Although intrathoracic lipomas are histologically benign, careful observation and follow-up are crucial due to the possibility of recurrence.

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Intraoperative Anesthetic Management of Patients Undergoing Glomus Tumor Resection Using a Low-Dose Isoflurane-Fentanyl Technique

Skull Base ◽

10.1055/s-2008-1058975 ◽

1994 ◽

Vol 4 (02) ◽

pp. 82-86

Author(s):

W.S. Jellish ◽

J. Murdoch ◽

J. Leonetti

Keyword(s):

Low Dose ◽

Glomus Tumor ◽

Anesthetic Management ◽

Tumor Resection

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The Unpredictability of Idiopathic Membranous Nephropathy: An Illustrative Case Report

Case Reports in Nephrology and Dialysis ◽

10.1159/000489773 ◽

2018 ◽

Vol 8 (2) ◽

pp. 103-106

Author(s):

Murray L. Levin ◽

Shubhada Ahya

Keyword(s):

Nephrotic Syndrome ◽

Case Report ◽

Complete Remission ◽

Female Patient ◽

Membranous Nephropathy ◽

Low Dose ◽

Conservative Therapy ◽

Current Knowledge ◽

Illustrative Case ◽

Cytomegalovirus Colitis

The case of a female patient with primary membranous nephropathy is presented. She was treated with corticosteroids and chlorambucil after conservative therapy had failed and went into remission for 5 years. Her nephrotic syndrome recurred but did not respond to the same regimen. She had another complete remission after treatment with corticosteroids and cyclosporine, but the nephrosis recurred after 7 years. Again, she failed to respond with retreatment of steroids plus cyclosporine. She was treated with alternate-day steroid plus mycophenolate and, once again, had a complete remission. She was maintained on low-dose mycophenolate for 7 more years. The mycophenolate had to be discontinued because of cytomegalovirus colitis. Treatment with ganciclovir abolished the colitis. She is still in remission 10 years later. The case is discussed with regard to current knowledge of the immune pathogenesis of membranous nephropathy as well as the unknowns of the immunogenesis of the disease.

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Short-term preoperative treatment of macroprolactinomas by dopamine agonists

Journal of Neurosurgery ◽

10.3171/jns.1987.67.6.0807 ◽

1987 ◽

Vol 67 (6) ◽

pp. 807-815 ◽

Cited By ~ 39

Author(s):

Rudolf Fahlbusch ◽

Michael Buchfelder ◽

Uwe Schrell

Keyword(s):

Dopamine Agonists ◽

Low Dose ◽

Tumor Resection ◽

Residual Tumor ◽

Cystic Tumor ◽

Preoperative Treatment ◽

Short Term ◽

Content Type ◽

Transsphenoidal Microsurgery ◽

Injectable Form

✓ During a period of 3 years, 25 patients with intra- and extrasellar macroprolactinomas were pretreated with dopamine agonists for a period of 2 to 6½ weeks prior to transsphenoidal microsurgical tumor resection. Dopamine agonists were administered orally to 17 patients, intramuscularly to three patients, and both orally and intramuscularly to five patients. Repeated computerized tomography (CT) examinations revealed that all neoplasms except one cystic tumor were reduced in size during the course of dopamine-agonist administration. No complications attributable to medical pretreatment were observed. Tumor shrinkage increased the efficacy of surgery, especially in cases with considerable extrasellar extension of the adenomas. Within 3 months following adenomectomy, prolactin levels were adjusted to normal levels in 19 patients by additional low-dose treatment with dopamine agonists. Thin-collimation CT assessments performed at least 3 months after surgery showed no evidence of residual tumor tissue in 23 patients. It is concluded that administration of dopamine agonists for some weeks prior to surgery is a useful adjunct to transsphenoidal microsurgery for macroprolactinomas. The new injectable form of bromocriptine is particularly valuable for this purpose.

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