Extraneuraxial Hemangioblastoma:

Michele Bisceglia, Lucia A. Muscarella, Carlos A. Galliani, Nina Zidar, David Ben-Dor, Gianandrea Pasquinelli, Annamaria La Torre, Angelo Sparaneo, Julie Fanburg-Smith, Janez Lamovec, Michal Michal, Carlos E. Bacchi

Research output: Contribution to journalReview article

2 Citations (Scopus)

Abstract

Extraneuraxial hemangioblastoma occurs in nervous paraneuraxial structures, somatic tissues, and visceral organs, as part of von Hippel-Lindau disease (VHLD) or in sporadic cases. The VHL gene plausibly plays a key role in the initiation and tumorigenesis of both central nervous system and extraneuraxial hemangioblastoma, therefore, the underlying molecular and genetic mechanisms of the tumor growth are initially reviewed. The clinical criteria for the diagnosis of VHLD are summarized, with emphasis on the distinction of sporadic hemangioblastoma from the form fruste of VHLD (eg, hemangioblastoma- only VHLD). The world literature on the topic of extraneuraxial hemangioblastomas has been comprehensively reviewed with 200 cases reported to date: up to 140 paraneuraxial, mostly of proximal spinal nerve roots, and 65 peripheral, 15 of soft tissue, 6 peripheral nerve, 5 bone, and 39 of internal viscera, including 26 renal and 13 nonrenal. A handful of possible yet uncertain cases from older literature are not included in this review. The clinicopathologic features of extraneuraxial hemangioblastoma are selectively presented by anatomic site of origin, and the differential diagnosis is emphasized in these subsets. Reference is made also to 10 of the authors' personal cases of extraneuraxial hemangioblastomas, which include 4 paraneuraxial and 6 peripheral (2 soft tissue hemangioblastoma and 4 renal).

Original languageEnglish (US)
Pages (from-to)197-215
Number of pages19
JournalAdvances in Anatomic Pathology
Volume25
Issue number3
DOIs
StatePublished - Jan 1 2018

Fingerprint

Hemangioblastoma
von Hippel-Lindau Disease
Kidney
Viscera
Spinal Nerve Roots
Peripheral Nerves
Molecular Biology
Carcinogenesis
Differential Diagnosis
Central Nervous System
Bone and Bones

All Science Journal Classification (ASJC) codes

  • Anatomy
  • Pathology and Forensic Medicine

Cite this

Bisceglia, M., Muscarella, L. A., Galliani, C. A., Zidar, N., Ben-Dor, D., Pasquinelli, G., ... Bacchi, C. E. (2018). Extraneuraxial Hemangioblastoma: Advances in Anatomic Pathology, 25(3), 197-215. https://doi.org/10.1097/PAP.0000000000000176
Bisceglia, Michele ; Muscarella, Lucia A. ; Galliani, Carlos A. ; Zidar, Nina ; Ben-Dor, David ; Pasquinelli, Gianandrea ; Torre, Annamaria La ; Sparaneo, Angelo ; Fanburg-Smith, Julie ; Lamovec, Janez ; Michal, Michal ; Bacchi, Carlos E. / Extraneuraxial Hemangioblastoma:. In: Advances in Anatomic Pathology. 2018 ; Vol. 25, No. 3. pp. 197-215.
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abstract = "Extraneuraxial hemangioblastoma occurs in nervous paraneuraxial structures, somatic tissues, and visceral organs, as part of von Hippel-Lindau disease (VHLD) or in sporadic cases. The VHL gene plausibly plays a key role in the initiation and tumorigenesis of both central nervous system and extraneuraxial hemangioblastoma, therefore, the underlying molecular and genetic mechanisms of the tumor growth are initially reviewed. The clinical criteria for the diagnosis of VHLD are summarized, with emphasis on the distinction of sporadic hemangioblastoma from the form fruste of VHLD (eg, hemangioblastoma- only VHLD). The world literature on the topic of extraneuraxial hemangioblastomas has been comprehensively reviewed with 200 cases reported to date: up to 140 paraneuraxial, mostly of proximal spinal nerve roots, and 65 peripheral, 15 of soft tissue, 6 peripheral nerve, 5 bone, and 39 of internal viscera, including 26 renal and 13 nonrenal. A handful of possible yet uncertain cases from older literature are not included in this review. The clinicopathologic features of extraneuraxial hemangioblastoma are selectively presented by anatomic site of origin, and the differential diagnosis is emphasized in these subsets. Reference is made also to 10 of the authors' personal cases of extraneuraxial hemangioblastomas, which include 4 paraneuraxial and 6 peripheral (2 soft tissue hemangioblastoma and 4 renal).",
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Bisceglia, M, Muscarella, LA, Galliani, CA, Zidar, N, Ben-Dor, D, Pasquinelli, G, Torre, AL, Sparaneo, A, Fanburg-Smith, J, Lamovec, J, Michal, M & Bacchi, CE 2018, 'Extraneuraxial Hemangioblastoma:', Advances in Anatomic Pathology, vol. 25, no. 3, pp. 197-215. https://doi.org/10.1097/PAP.0000000000000176

Extraneuraxial Hemangioblastoma: / Bisceglia, Michele; Muscarella, Lucia A.; Galliani, Carlos A.; Zidar, Nina; Ben-Dor, David; Pasquinelli, Gianandrea; Torre, Annamaria La; Sparaneo, Angelo; Fanburg-Smith, Julie; Lamovec, Janez; Michal, Michal; Bacchi, Carlos E.

In: Advances in Anatomic Pathology, Vol. 25, No. 3, 01.01.2018, p. 197-215.

Research output: Contribution to journalReview article

TY - JOUR

T1 - Extraneuraxial Hemangioblastoma:

AU - Bisceglia, Michele

AU - Muscarella, Lucia A.

AU - Galliani, Carlos A.

AU - Zidar, Nina

AU - Ben-Dor, David

AU - Pasquinelli, Gianandrea

AU - Torre, Annamaria La

AU - Sparaneo, Angelo

AU - Fanburg-Smith, Julie

AU - Lamovec, Janez

AU - Michal, Michal

AU - Bacchi, Carlos E.

PY - 2018/1/1

Y1 - 2018/1/1

N2 - Extraneuraxial hemangioblastoma occurs in nervous paraneuraxial structures, somatic tissues, and visceral organs, as part of von Hippel-Lindau disease (VHLD) or in sporadic cases. The VHL gene plausibly plays a key role in the initiation and tumorigenesis of both central nervous system and extraneuraxial hemangioblastoma, therefore, the underlying molecular and genetic mechanisms of the tumor growth are initially reviewed. The clinical criteria for the diagnosis of VHLD are summarized, with emphasis on the distinction of sporadic hemangioblastoma from the form fruste of VHLD (eg, hemangioblastoma- only VHLD). The world literature on the topic of extraneuraxial hemangioblastomas has been comprehensively reviewed with 200 cases reported to date: up to 140 paraneuraxial, mostly of proximal spinal nerve roots, and 65 peripheral, 15 of soft tissue, 6 peripheral nerve, 5 bone, and 39 of internal viscera, including 26 renal and 13 nonrenal. A handful of possible yet uncertain cases from older literature are not included in this review. The clinicopathologic features of extraneuraxial hemangioblastoma are selectively presented by anatomic site of origin, and the differential diagnosis is emphasized in these subsets. Reference is made also to 10 of the authors' personal cases of extraneuraxial hemangioblastomas, which include 4 paraneuraxial and 6 peripheral (2 soft tissue hemangioblastoma and 4 renal).

AB - Extraneuraxial hemangioblastoma occurs in nervous paraneuraxial structures, somatic tissues, and visceral organs, as part of von Hippel-Lindau disease (VHLD) or in sporadic cases. The VHL gene plausibly plays a key role in the initiation and tumorigenesis of both central nervous system and extraneuraxial hemangioblastoma, therefore, the underlying molecular and genetic mechanisms of the tumor growth are initially reviewed. The clinical criteria for the diagnosis of VHLD are summarized, with emphasis on the distinction of sporadic hemangioblastoma from the form fruste of VHLD (eg, hemangioblastoma- only VHLD). The world literature on the topic of extraneuraxial hemangioblastomas has been comprehensively reviewed with 200 cases reported to date: up to 140 paraneuraxial, mostly of proximal spinal nerve roots, and 65 peripheral, 15 of soft tissue, 6 peripheral nerve, 5 bone, and 39 of internal viscera, including 26 renal and 13 nonrenal. A handful of possible yet uncertain cases from older literature are not included in this review. The clinicopathologic features of extraneuraxial hemangioblastoma are selectively presented by anatomic site of origin, and the differential diagnosis is emphasized in these subsets. Reference is made also to 10 of the authors' personal cases of extraneuraxial hemangioblastomas, which include 4 paraneuraxial and 6 peripheral (2 soft tissue hemangioblastoma and 4 renal).

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Bisceglia M, Muscarella LA, Galliani CA, Zidar N, Ben-Dor D, Pasquinelli G et al. Extraneuraxial Hemangioblastoma: Advances in Anatomic Pathology. 2018 Jan 1;25(3):197-215. https://doi.org/10.1097/PAP.0000000000000176