scholarly journals Sporadic supratentorial hemangioblastoma with meningeal affection: A case report and literature review

2021 ◽  
Vol 12 ◽  
pp. 394
Author(s):  
Juan Francisco Sánchez-Ortega ◽  
Marta Claramonte ◽  
Mónica Martín ◽  
Juan Calatayud-Pérez
Keyword(s):  
Blood Vessels ◽  
Case Series ◽  
Imaging Study ◽  
Vascular Involvement ◽  
Vascular Tumors ◽  
En Bloc ◽  
Von Hippel Lindau ◽  
Meningeal Involvement ◽  
Adjacent Structures ◽  
Superior Longitudinal Sinus

Background: Hemangioblastomas are vascular tumors, of benign behavior, that originate in the central nervous system. Supratentorial hemangioblastomas are extremely rare and are generally associated with Von Hippel-Lindau disease (VHL). The involvement of structures by contiguity, such as blood vessels or meninges, is something exceptional. There are few references in the literature of supratentorial hemangioblastomas with meningeal involvement and most of them are described in reports or small case series. Methods: We performed a systematic review of the literature to observe the characteristics of patients with supratentorial hemangioblastomas with meningeal involvement. In addition, we present the case of a 43-year-old male patient with a sporadic supratentorial hemangioblastoma with both, meningeal and vascular involvement that recurred years after treatment. Results: The patients presented supratentorial tumors with meningeal involvement, we had a 1.2:1 ratio male-female distribution. The mean age was 50 years. Most tumors were located in the cerebral hemispheres, the lobe affected most frequently was the frontal lobe. About 67% of the cases were sporadic and only 21% were related to VHL disease. There were three cases of recurrence after surgery. Conclusion: Supratentorial hemangioblastomas are extremely rare lesions. It is rare for supratentorial hemangioblastomas to invade adjacent structures such as blood vessels or meninges, however, when this happens, it is recommended a preoperative vascular imaging study, especially in parasagittal hemangioblastomas where superior longitudinal sinus may be involved. In these cases, en bloc surgical resection is difficult and the probability of recurrence is higher. Although clinical trials and studies with a greater casuistry are necessary to establish guidelines for the management of supratentorial hemangioblastomas, nowadays, contribution of new cases is useful for understanding this pathology.

Resection of a Medulla Oblongata Hemangioblastoma: 2-Dimensional Operative Video

2021 ◽  
Author(s):  
Walid Ibn Essayed ◽  
Ossama Al-Mefty
Keyword(s):  
Venous Drainage ◽  
Radical Resection ◽  
Vascular Tumors ◽  
En Bloc ◽  
Von Hippel Lindau ◽  
Suboccipital Craniotomy ◽  
Von Hippel Lindau Disease ◽  
The Central Nervous System ◽  
Technical Principles ◽  
Dorsal Medulla

Abstract Hemangioblastomas are benign vascular tumors that can be sporadic or multiple, as part of Von Hippel-Lindau disease. They develop at any level of the central nervous system, with a predilection for the dorsal medulla among brainstem locations. Radical resection of the solid portion of the tumor is the best treatment option.1,2 The resection should be en bloc to avoid uncontrollable intraoperative hemorrhage hindering safe dissection. Preservation of the venous drainage during the progressive dissection of the tumor of the surrounding structures and interruption of numerous small arterial feeders is a tenet for safe surgical resection.3 Once the tumor is completely disconnected, the large draining veins can be coagulated, and the tumor removed. We demonstrate these technical principles in the surgery of a 30-yr-old female with an exophytic hemangioblastoma from the dorsal medulla obstructing the fourth ventricle outflow. We demonstrate the resection of this lesion through a suboccipital craniotomy in a sitting position.4 The patient consented to the surgery and publication of images. Image at 1:26 from Kadri and Al-Mefty,4 by permission from the Congress of Neurological Surgeons.

scholarly journals Inactivation of the Arylhydrocarbon Receptor Nuclear Translocator (Arnt) Suppresses von Hippel-Lindau Disease-Associated Vascular Tumors in Mice

2005 ◽  
Vol 25 (8) ◽  
pp. 3163-3172 ◽  
Author(s):  
Erinn B. Rankin ◽  
Debra F. Higgins ◽  
Jacqueline A. Walisser ◽  
Randall S. Johnson ◽  
Christopher A. Bradfield ◽  
...  
Keyword(s):  
Transcription Factors ◽  
Germ Line ◽  
Hypoxia Inducible Factor ◽  
Suppressor Gene ◽  
Vascular Tumors ◽  
Von Hippel Lindau ◽  
Von Hippel Lindau Disease ◽  
Arylhydrocarbon Receptor ◽  
Vhl Disease ◽  
Endothelial Growth Factor

ABSTRACT Patients with germ line mutations in the VHL tumor suppressor gene are predisposed to the development of highly vascularized tumors within multiple tissues. Loss of pVHL results in constitutive activation of the transcription factors HIF-1 and HIF-2, whose relative contributions to the pathogenesis of the VHL phenotype have yet to be defined. In order to examine the role of HIF in von Hippel-Lindau (VHL)-associated vascular tumorigenesis, we utilized Cre-loxP-mediated recombination to inactivate hypoxia-inducible factor-1α (Hif-1α) and arylhydrocarbon receptor nuclear translocator (Arnt) genes in a VHL mouse model of cavernous liver hemangiomas and polycythemia. Deletion of Hif-1α did not affect the development of vascular tumors and polycythemia, nor did it suppress the increased expression of vascular endothelial growth factor (Vegf) and erythropoietin (Epo). In contrast, phosphoglycerokinase (Pgk) expression was substantially decreased, providing evidence for target gene-dependent functional redundancy between different Hif transcription factors. Inactivation of Arnt completely suppressed the development of hemangiomas, polycythemia, and Hif-induced gene expression. Here, we demonstrate genetically that the development of VHL-associated vascular tumors in the liver depends on functional ARNT. Furthermore, we provide evidence that individual HIF transcription factors may play distinct roles in the development of specific VHL disease manifestations.

scholarly journals Long-term follow-up after colorectal endoscopic submucosal dissection in 182 cases

2021 ◽  
Vol 09 (02) ◽  
pp. E258-E262
Author(s):  
Christian Suchy ◽  
Moritz Berger ◽  
Ingo Steinbrück ◽  
Tsuneo Oyama ◽  
Naohisa Yahagi ◽  
...  
Keyword(s):  
Case Series ◽  
En Bloc Resection ◽  
Bloc Resection ◽  
Published Data ◽  
Low Grade ◽  
Recurrence Rates ◽  
En Bloc ◽  
Long Term Follow Up

Abstract Background and study aims We previously reported a case series of our first 182 colorectal endoscopic submucosal dissections (ESDs). In the initial series, 155 ESDs had been technically feasible, with 137 en bloc resections and 97 en bloc resections with free margins (R0). Here, we present long-term follow-up data, with particular emphasis on cases where either en bloc resection was not achieved or en bloc resection resulted in positive margins (R1). Patients and methods Between September 2012 and October 2015, we performed 182 consecutive ESD procedures in 178 patients (median size 41.0 ± 17.4 mm; localization rectum vs. proximal rectum 63 vs. 119). Data on follow-up were obtained from our endoscopy database and from referring physicians. Results Of the initial cohort, 11 patients underwent surgery; follow-up data were available for 141 of the remaining 171 cases (82,5 %) with a median follow-up of 2.43 years (range 0.15–6.53). Recurrent adenoma was observed in 8 patients (n = 2 after margin positive en bloc ESD; n = 6 after fragmented resection). Recurrence rates were lower after en bloc resection, irrespective of involved margins (1.8 vs. 18,2 %; P < 0.01). All recurrences were low-grade adenomas and could be managed endoscopically. Conclusions The rate of recurrence is low after en bloc ESD, in particular if a one-piece resection can be achieved. Recurrence after fragmented resection is comparable to published data on piecemeal mucosal resection.

Pulmonary Hypertension Associated With Myocardial Amyloid Degeneration: a Case Report

2021 ◽  
Author(s):  
Lu Yan ◽  
Qixian Zeng ◽  
Changming Xiong ◽  
Zhihui Zhao ◽  
Qing Zhao ◽  
...  
Keyword(s):  
Heart Failure ◽  
Pulmonary Hypertension ◽  
Blood Vessels ◽  
Amyloid Fibrils ◽  
Right Heart Failure ◽  
Pulmonary Artery Hypertension ◽  
Pulmonary Vasculature ◽  
Vascular Involvement ◽  
Right Heart ◽  
Protein Deposits

Abstract Background: There is very little literature on Pulmonary hypertension associated with myocardial amyloid degeneration. At present, only 10 cases pulmonary hypertension cased by amyloid protein deposits in the pulmonary blood vessels have been reported by Eder et al. We reported a case that the patient was pulmonary artery hypertension combined with myocardial amyloid change. It’s aim to claims that pulmonary hypertension is most likely caused by amyloid fibrin deposition in pulmonary blood vessels.Case presentation: We report a case of a 65-year-old male patient with with AL and ATTR combined type amyloidosis who developed right heart failure because of severe pulmonary hypertension. Pulmonary hypertension due to deposition of amyloid in the pulmonary vasculature is an uncommon finding; however, it should be considered in cases of unexplained pulmonary hypertension in patients with amyloidosis.Conclusion: we present a men with amyloidosis who developed dyspnea and right heart failure and was diagnosed with pulmonary hypertension, most probably secondary to pulmonary vascular involvement by amyloid fibrils.

scholarly journals Clinical suspicion and parathyroid carcinoma management

2006 ◽  
Vol 124 (1) ◽  
pp. 42-44 ◽  
Author(s):  
Fabio Luiz de Menezes Montenegro ◽  
Marcos Roberto Tavares ◽  
Marcelo Doria Durazzo ◽  
Claudio Roberto Cernea ◽  
Anói Castro Cordeiro ◽  
...  
Keyword(s):  
Primary Hyperparathyroidism ◽  
Secondary Hyperparathyroidism ◽  
Parathyroid Carcinoma ◽  
Clinical Signs ◽  
Neck Surgery ◽  
Cross Sectional Study ◽  
Clinical Suspicion ◽  
En Bloc ◽  
Cross Sectional ◽  
Adjacent Structures

CONTEXT AND OBJECTIVE: Adequate management of parathyroid carcinoma apparently relates to the surgeon’s ability to identify it at the first operation. The objective of this paper was to evaluate the role of clinical suspicion in the management of parathyroid carcinoma. DESIGN AND SETTING: Retrospective analysis of parathyroid carcinoma patients treated in Department of Head and Neck Surgery, Faculdade de Medicina da Universidade de São Paulo. METHODS: Cross-sectional study of 143 patients who underwent surgery from 1995 to 2000, due to hyperparathyroidism. These cases were reviewed to ascertain whether preoperative and intraoperative suspicion of parathyroid carcinoma were helpful during the operation, and which factors demonstrated the suspicion of cancer best. RESULTS: Among 66 patients with primary hyperparathyroidism there were four cases of parathyroid carcinoma (6.1%), and one case was found in secondary hyperparathyroidism (1.3%). Palpable nodules were found in five patients with primary hyperparathyroidism, four of them with parathyroid carcinoma. Preoperative levels of calcium in primary hyperparathyroidism with cancer patients varied from 12.0 mg/dl to 18.2 mg/dl. Two patients had gross macroscopic spread of the tumor to adjacent structures. Except for one patient, with extensive disease, tumors were resected en bloc. In secondary hyperparathyroidism, parathyroid carcinoma was found in a fifth mediastinal gland. One atypical adenoma was observed. CONCLUSIONS: High levels of calcium, palpable tumors and adherence to close structures are more common in parathyroid carcinoma. These clinical signs may be helpful for decision-making during parathyroid surgery.

Nonvascularized Toe Joint Transfers to the Hand in Young Children: Technique Revisited

Hand ◽  
2020 ◽  
pp. 155894472094824
Author(s):  
Jeffrey G. Trost ◽  
Matthew Kaufman ◽  
David T. Netscher
Keyword(s):  
Case Series ◽  
Extensor Tendon ◽  
Finger Joint ◽  
Growth Potential ◽  
Microvascular Reconstruction ◽  
Practical Consideration ◽  
En Bloc ◽  
Joint Reconstruction ◽  
Congenital Deformities ◽  
Small Joint

Background: Small joint reconstruction of the hand poses a significant challenge, especially in children where both functional motion and preservation of the epiphysis are desired. Auto-transplantation of whole joints is the only way to reconstruct a functional joint that maintains growth potential. Historically, nonvascularized toe-to-finger joint transfer has been criticized for high rates of avascular necrosis and joint dissolution, while vascularized transfers seemingly resulted in increased durability of the joint space and epiphysis. However, certain populations remain poor candidates for microvascular reconstruction, such as those with congenital deformities or sequelae from trauma or infection. In our case series, we demonstrate that a simplified nonvascularized surgical technique and careful patient selection can produce stable, functional joints. Methods: Nonvascularized toe-to-finger joint transfer was performed in 3 children between the ages of 4 and 6. Reconstructed joints included 2 proximal interphalangeal (PIP) joints and 1 metacarpophalangeal (MCP) joint. Donor grafts consisted of second toe PIP joints harvested en bloc to include the epiphysis of the middle phalanx, collateral ligaments, volar plate, and a diamond-shaped island of extensor tendon with its central slip attachment. Results: Follow-up ranged from 7 to 29 months. Each patient demonstrated functional improvements in joint motion and stability. Postoperative radiographs confirmed adequate joint alignment and persistence of the joint spaces. Epiphyseal closure was observed in 1 patient as early as 25 months postoperatively. Conclusions: Nonvascularized joint transfer should remain a practical consideration for small joint reconstruction of the hand in certain pediatric patients.

scholarly journals Varied presentations of cutaneous vasculitis: a case series

2020 ◽  
Vol 8 (8) ◽  
pp. 3066
Author(s):  
Pinky Gupta ◽  
Shweta Ganorkar ◽  
Surekha Bhalekar ◽  
Rajiv Rao
Keyword(s):  
Blood Vessels ◽  
Histopathological Examination ◽  
Wide Spectrum ◽  
Systemic Disease ◽  
Case Series ◽  
Clinical Severity ◽  
Cutaneous Vasculitis ◽  
Venous Stasis ◽  
Duration Of Symptoms ◽  
Organ Systems

Vasculitis involves a wide spectrum of clinicopathological process with reactive damage to the involved blood vessels. There is loss of vessel integrity instigating haemorrhage & luminal compromise leading to ischemia and necrosis of the tissue supplied by the involved vessels. It may affect varied size and type of blood vessels at different locations. It may be primary or secondary to systemic disease. It may involve a single organ like skin or may involve different organ systems at the same time. This case series include six cases of cutaneous vasculitis affecting different organs with varied presentations. Skin biopsies of six patients with unusual presentations were studied. Their complete history, physical examinations, laboratory investigations including serology were analysed and correlated with histopathological findings. The patients presented with different duration of symptoms varying from as short as 15 days to 1 year. Skin lesions were present in all cases while cardiac manifestation was seen in one. Serology and autoimmune disease markers were negative in all cases except one. However, histopathological features were in concordance with the clinical diagnosis of vasculitis. They were further classified as vasculitis secondary to Churg Strauss syndrome, venous stasis, Henoch Schonlein purpura or leucocytoclastic vasculitis.Vasculitis though a rare disease may manifest as an acute or chronic condition. It needs timely diagnosis by histopathological examination to aid in further management. It is important to assess the clinical severity in primary and secondary vasculitis, as it determines morbidity and mortality.

scholarly journals Karakteristik radiograf kista dentigerous dengan menggunakan CBCT-scan

2020 ◽  
Vol 4 (2) ◽  
pp. 15
Author(s):  
Berty Pramatika ◽  
Aga Satria Nurrachman ◽  
Eha Renwi Astuti
Keyword(s):  
Case Report ◽  
Surgical Planning ◽  
Dentigerous Cyst ◽  
Three Dimensional ◽  
Case Series ◽  
Impacted Tooth ◽  
Adjacent Structures

Objectives: The aim of this report which contains three case series is to describe the radiographic characteristic of dentigerous cyst using CBCT. Case Report: In the case presented here, all of the three patients had dentigerous cyst developing in impacted tooth, but did not have the same symptoms. CBCT radiography examination was carried out to find out the margin of the cortical extension, the diameter of the lesion, and the relations between the lesion and adjacent structures. The result of CBCT examination shows there is a variation of radiograph characteristics of dentigerous cyst among the three patients. Conclusion: CBCT is a very useful complementary tool for diagnosis and surgical planning in cases of dentigerous cyst, because three-dimensional viewing of the structures offers greater accuracy in lesion identification.

En Bloc Resection of the Duodenum for Locally Advanced Right Colon Adenocarcinoma

2007 ◽  
Vol 73 (10) ◽  
pp. 1063-1066 ◽  
Author(s):  
Ahmad N. Hakimi ◽  
David K. Rosing ◽  
Bruce E. Stabile ◽  
Beverley A. Petrie
Keyword(s):  
Colon Cancer ◽  
Locally Advanced ◽  
En Bloc Resection ◽  
Bloc Resection ◽  
Morbidity And Mortality ◽  
Right Colon ◽  
En Bloc ◽  
Adjacent Structures ◽  
Duodenal Invasion

Direct invasion of colorectal adenocarcinoma into adjacent structures occurs frequently, but only rarely is the duodenum involved. This study was undertaken to assess the safety and efficacy of en bloc resection of locally advanced right colon carcinoma invading the duodenum. A retrospective review of 49 patients with locally advanced colon cancer, surgically managed between 2000 and 2005, was performed. Forty-six patients underwent en bloc resection of colon and adjacent organs not involving the duodenum. Three patients with duodenal invasion underwent en bloc partial duodenectomy. The mean operative blood loss, length of stay, postoperative morbidity, and mortality compare favorably between these two groups of patients. Of the 46 patients with en bloc resection of other organs, 27 are alive at 12 to 60 months follow up. Two patients with duodenal invasion are alive without recurrence at 15 and 20 months follow up. En bloc resection of colon cancer invading the duodenum can be performed safely because morbidity and mortality rates are comparable to those attending extended resections of other locally advanced colon carcinomas. Overall survival in patients who underwent surgery with curative intent justifies en bloc duodenal resection in selected patients.

Sign in / Sign up

Export Citation Format

Share Document