Cerebral Hemangioblastoma Without Von Hippel-Lindau Syndrome: A Report of 6 Cases

2020 ◽  
pp. 106689692093399
Author(s):  
Limei Qu ◽  
Chuanqi Lv ◽  
Tiefeng Ji ◽  
Yinping Wang ◽  
Jinlu Yu
Keyword(s):  
Parietal Lobe ◽  
Clinical Manifestations ◽  
Pathological Examination ◽  
Cystic Tumor ◽  
Mixed Tumor ◽  
Wild Type ◽  
Von Hippel Lindau ◽  
Imaging Characteristics ◽  
Von Hippel Lindau Syndrome

Background. Hemangioblastoma occurs mainly in the cerebellum and rarely in the cerebrum. Objective. The present study aimed to analyze the clinical manifestations and radiological and pathological features of cerebral hemangioblastoma, and to improve the recognition of this tumor and avoid misdiagnosis. Methods. The characteristics of 6 patients with cerebral hemangioblastoma were analyzed, and a retrospective review of cerebral hemangioblastoma reported in the literature was performed. Results. All 6 patients were female, aged from 22 to 70 years (55 years on average), and all cases were wild-type sporadic, in which 4 cases occurred in the frontal lobe and 2 cases occurred in the parietal lobe. Imaging revealed a solid tumor in 4 cases, a cystic tumor in 1 case, and a mixed tumor in 1 case. Microscopically, the morphology and immunophenotype of tumor cells were not different from those of classical hemangioblastoma. All 6 patients survived tumor free during the follow-up period. Conclusions. Cerebral hemangioblastoma often simulates the imaging characteristics of meningioma or glioma. Enough attention should be paid to differential diagnosis before the operation, and exact diagnosis relies on the pathological examination.

Intracranial parenchymal schwannoma

1993 ◽  
Vol 79 (2) ◽  
pp. 217-222 ◽  
Author(s):  
Gian Piero Casadei ◽  
Takashi Komori ◽  
Bernd W. Scheithauer ◽  
Gary M. Miller ◽  
Joseph E. Parisi ◽  
...  
Keyword(s):  
Parietal Lobe ◽  
Clinical Course ◽  
Cystic Degeneration ◽  
Resonance Imaging ◽  
Imaging Studies ◽  
Content Type ◽  
Imaging Characteristics ◽  
Benign Nature ◽  
Peripheral Enhancement

✓ The clinical, radiological, and pathological features of nine cases of intracranial parenchymal schwannoma are described. The clinical course in four patients 23 years of age or younger mirrored the indolent nature of this neoplasm. Imaging studies included computerized tomography in eight patients and magnetic resonance imaging in three. The lesions were well demarcated with only mild surrounding edema. Five tumors were deep within the temporoparieto-occipital region, three were in the cerebellum, and one lay peripherally in the parietal lobe. Over two-thirds of the nine tumors were either cystic (five) or contained areas of cystic degeneration (two). One lesion was frankly hemorrhagic. A variety of imaging characteristics and contrast enhancement patterns were observed, including those of a cyst with a mural nodule and peripheral enhancement. Of the four solid neoplasms, two enhanced homogeneously while the other two demonstrated heterogeneous enhancement. Six tumors were resected totally. The follow-up period ranging from 2 months to 2 years has shown no recurrences. Microscopically, immunohistochemically, and ultrastructurally, the tumors were indistinguishable from peripheral schwannomas. A possible mechanism underlying the histogenesis of these rare lesions is discussed. The importance of recognizing this tumor is stressed, particularly in younger patients, given its benign nature, radiological resemblance to other tumors such as pilocytic astrocytoma, and favorable response to resection.

Preliminary Exploration of the Diagnosis and Treatment of Skull-Based Chondromyxoid Fibromas

2017 ◽  
Vol 15 (3) ◽  
pp. 270-277
Author(s):  
Kangmin He ◽  
Shize Jiang ◽  
Xin Zhang ◽  
Ying Mao ◽  
Wei Zhu ◽  
...  
Keyword(s):  
Clinical Manifestations ◽  
Treatment Strategies ◽  
Diagnosis And Treatment ◽  
Benign Tumors ◽  
Symptom Duration ◽  
Study Cohort ◽  
Imaging Characteristics ◽  
Subtotal Removal ◽  
Base Of The Skull

Abstract BACKGROUND Chondromyxoid fibromas (CMFs) are benign tumors that occur rarely in the skull base. OBJECTIVE To conduct a preliminary exploration of the diagnosis and treatment of cranial CMFs. METHODS A retrospective analysis of 19 cases of CMFs in the base of the skull between 2009 and 2014 in our hospital was conducted. The clinical manifestations, imaging characteristics, pathology, treatment strategies, and outcomes were examined. RESULTS The study cohort included 7 women (36.8%) and 12 men (63.2%), and symptom duration ranged from 1 mo to 5 yr. Of the 19 intracranial CMF cases examined, 15 (78.9%) conformed with the diagnostic criteria for extracranial CMF. Resection operations yielded subtotal removal of 13 tumors (68.4%) and partial removal of 6 tumors (31.6%). Postoperative pathological analysis demonstrated that the tumors were characterized by spindle-shaped or stellate cells arranged in a myxoid matrix without mitoses or permeation. Follow-up (range 2-7.3 yr; mean, 4.4 ± 1.7 yr) revealed that symptoms improved postoperatively in 15 cases (78.9%), were maintained in 2 cases (10.5%), and worsened in 2 cases (10.5%). Imaging follow-up revealed that residual tumors were stable in 18 cases (94.7%) and enlarged in 1 case (5.3%). CONCLUSION An accurate diagnosis should involve comprehensive consideration of clinical, radiological, and pathological features. The treatment strategy for CMFs consists of maximizing tumor removal while protecting adjacent key structures. Postoperative stereotactic radiotherapy is appropriate for residual tumors.

scholarly journals MP80-11 CLINICAL, SURGICAL, PATHOLOGICAL AND FOLLOW-UP FEATURES OF KIDNEY CANCER IN PATIENTS WITH VON HIPPEL-LINDAU SYNDROME

2020 ◽  
Vol 203 ◽  
pp. e1229
Author(s):  
Umberto Capitanio* ◽  
Erdem Selcuk ◽  
Isaline Rowe ◽  
Kara Onder ◽  
Eduard Roussel ◽  
...  
Keyword(s):  
Kidney Cancer ◽  
Von Hippel Lindau ◽  
Von Hippel Lindau Syndrome

scholarly journals Diagnosis of von Hippel-Lindau Syndrome Following Endoscopic Ultrasound of the Pancreas

2009 ◽  
Vol 1 (1) ◽  
pp. 41-42
Author(s):  
Jennifer Cannon ◽  
Paul G Gauger ◽  
Gerard M Doherty ◽  
James M Scheiman
Keyword(s):  
Endoscopic Ultrasound ◽  
Islet Cell ◽  
Clinical Presentation ◽  
Clinical Manifestations ◽  
Islet Cell Tumors ◽  
Pancreatic Islet Cell ◽  
Von Hippel Lindau ◽  
History Of ◽  
Pancreatic Islet Cell Tumors ◽  
Von Hippel Lindau Syndrome

ABSTRACT Objective To report a case of von Hippel-Lindau (VHL) syndrome diagnosed by endoscopic ultrasound (EUS) of the pancreas in a patient with no family history of the disease and without any other clinical manifestations of VHL. Methods We describe the clinical presentation of the case and discuss its management. Results A 33-year-old woman was evaluated by EUS for possible pancreatic carcinoma. Based upon the sonographic appearance of her pancreas, VHL was suggested as a diagnosis and subsequently confirmed by genetic testing. The pancreatic lesions were treated surgically and pathology revealed pancreatic islet cell tumors. Conclusion This is the first report of a patient in which the first suggestion of a VHL diagnosis was made on EUS appearance of pancreatic lesions alone.

scholarly journals Outcomes of nephron sparing surgery and cortical sparing adrenalectomy in the management of Von Hippel–Lindau syndrome

2021 ◽  
Vol 27 (1) ◽  
Author(s):  
Selvin Theodore Jayanth ◽  
Partho Mukherjee ◽  
Arun Jacob Philip George ◽  
J. Chandrasingh ◽  
T. J. Nirmal ◽  
...  
Keyword(s):  
Tertiary Care ◽  
Adrenal Function ◽  
Nephron Sparing Surgery ◽  
Pancreatic Metastasis ◽  
Pancreatic Cysts ◽  
Adrenal Crisis ◽  
Von Hippel Lindau ◽  
Nephron Sparing ◽  
Von Hippel Lindau Syndrome

Abstract Background The management of Von Hippel–Lindau syndrome (VHLS) is multidisciplinary. The urologist is involved in treating the renal, epididymal tumors and often adrenal pheochromocytoma. Preservation of renal and adrenal function is a challenge. We present 17 cases of VHLS in a tertiary care center in South India. Methods A retrospective review of the patients who underwent surgical treatment under urology for VHLS from January 2009 to November 2018 was conducted. The demographic data, the spectrum of manifestation, treatment, change in glomerular filtration rate, adrenal insufficiency, and recurrence-free survival were analyzed. Results There were 17 patients diagnosed with VHLS. The median age of diagnosis was 39 years (range 23–41). The spectrum of clinical manifestation was: multifocal RCC (88%), pancreatic cysts/tumors (70%), cerebellar hemangioblastoma (59%), retinal angiomas (47%), epididymal cysts/tumors (47%), pheochromocytomas (41%), and spinal hemangiomas (30%). There were seven patients with ten pheochromocytoma lesions. Three underwent cortical sparing and seven total adrenalectomies; 13 patients underwent nephron sparing surgery (NSS), of which seven patients had bilateral tumors. The median duration of follow-up was 6.5 years (range 2–12 years). Following NSS, seven patients had a local recurrence, and one developed pancreatic metastasis. Two patients (11%) were lost to follow-up. Renal function was preserved in all patients at the last follow-up, and there was no postoperative adrenal crisis or mortality. Conclusion Nephron sparing surgery and cortical sparing adrenalectomy are the treatment of choice for multifocal RCC and pheochromocytomas in patients with VHLS providing good oncological outcomes and preservation of renal and adrenal function.

scholarly journals Pancreatic Leiomyosarcoma With Schistosomiasis Hematobia: A Case Report and Literature Review

2021 ◽  
Vol 11 ◽  
Author(s):  
Qiang Li ◽  
Daniel Staiculescu ◽  
Yurong Zhou ◽  
Jiang Chen
Keyword(s):  
Case Report ◽  
Tumor Resection ◽  
Clinical Manifestations ◽  
Malignant Neoplasm ◽  
The Body ◽  
Imaging Characteristics ◽  
Magnetic Resonance Imaging Mri ◽  
The Relationship ◽  
Ct And Mri

Pancreatic leiomyosarcoma (PL) is a very rare, malignant neoplasm with a very poor prognosis. Here, we examine a novel case of PL with schistosomiasis hematobia. The patient had been initially misdiagnosed by the first magnetic resonance imaging (MRI). The second imaging examination demonstrated an enlarged heterogeneous tumor mass in the body-tail of pancreas. Following image analysis, the patient underwent a pancreatectomy, splenectomy and lymph node dissections. Sixteen months after the tumor resection, follow-up computed tomography (CT) and MRI revealed tumor metastasis in the liver and lung. PL has non-specific clinical manifestations and imaging characteristics, making early diagnosis very challenging. When it is difficult to distinguish between benign and malignant pancreatic lesions, short-term imaging follow-up is preferred. In this case report, we discuss the relationship between PL and schistosomiasis hematobia.

scholarly journals Familial pineal tumours in two siblings

2015 ◽  
Vol 42 (S1) ◽  
pp. S45-S45
Author(s):  
FB Maroun ◽  
R Avery ◽  
E Walsh
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
Brain Tumours ◽  
Clinical Manifestations ◽  
The Other ◽  
Resonance Imaging ◽  
Von Hippel Lindau ◽  
Endocrine Neoplasia ◽  
Close Relationship

Background: The occurrence of familial brain tumours, particularly gliomas, hemangioblastomas in Von Hippel Lindau and other endocrine neoplasia, is well documented in the literature. On the other hand, familial pineal tumours are extremely rare and only a handful of cases have been reported. Methods and Results: Two female siblings presented at ages 12 and 15 with histories of progressive headaches. Neurological examination in each was completely normal. Magnetic Resonance Imaging confirmed the presence of cystic and solid lobulated pineal lesions with mild enhancement, consistent with pineocytoma, in both girls. Follow-up for 15 years in the first sibling and 4 years in the second showed no evolution in radiological or clinical manifestations. No active treatments have been carried out. Conclusion: The occurrence of familial pineal lesions raises the possibility of a close relationship between heredity and oncogenicity, and should be further explored.

scholarly journals P14.33 Adult brainstem gliomas in neurofibromatosis type 1 (NF1)

2019 ◽  
Vol 21 (Supplement_3) ◽  
pp. iii74-iii74
Author(s):  
J Guillamo ◽  
A Wang ◽  
F Ducray ◽  
S Barbarot ◽  
O Chinot ◽  
...  
Keyword(s):  
Medical Records ◽  
Median Overall Survival ◽  
Neurofibromatosis Type ◽  
Pathological Examination ◽  
Mri Contrast ◽  
High Grade Astrocytoma ◽  
Imaging Characteristics ◽  
Optic Pathways

Abstract BACKGROUND The brainstem is the second location of brain tumors after optic pathways in NF1. In NF1 children, brainstem gliomas are usually indolent and have a better prognosis than their counterparts in non NF1 children. In contrast, the natural history and prognosis of adult brainstem gliomas in NF1 are nearly unknown. MATERIAL AND METHODS We conducted a retrospective analysis of medical records and MRI of adult NF1 patients followed for a brainstem glioma in 8 centers over a 17 years period (2000–2017). Clinical and imaging characteristics, management and outcome were analyzed. RESULTS Twenty five patients were included in the study (13 males and 12 females) with a median age of 32 (range 17–58). The epicenter of the tumor was located into the pons n=13 (52%), the mesencephalon n=7 (28%), the medulla oblongata n=5 (20%). On MRI, contrast enhancement was seen in 19 tumors (76%). Pathological examination was available in 13 tumors (52%) and showed a high grade astrocytoma (III or IV) in 9 tumors. Five patients were asymptomatic, 3 remained asymptomatic during the follow-up (median follow-up: 86 months, range 22–124). Twenty patients were symptomatic with a median duration of symptom of 2.5 months (range 1–10) before diagnosis. Among these symptomatic patients, 15 died from tumor progression despite treatment with radiation therapy and or chemotherapy. The median overall survival of symptomatic patients was 36 months. CONCLUSION Brainstem gliomas are rare tumors in adults with NF1. Unlike children, adult brainstem gliomas seem to have an unexpected poor prognosis, suggesting the disease may be different in adulthood.

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