Collet-Sicard Syndrome Attributable to Extramedullary Plasmacytoma of the Jugular Foramen

Soliman Oushy, Christopher S. Graffeo, Avital Perry, Jonathan M. Morris, Matthew L. Carlson, Jamie Van Gompel

Research output: Contribution to journalArticle

1 Citation (Scopus)

Abstract

Background Collet-Sicard syndrome is a rare manifestation of skull base disease involving the jugular and hypoglossal foramina. We report the first case of Collet-Sicard attributable to extramedullary plasmacytoma—multiple myeloma (EP-MM) and the second case of EP-MM precipitating a jugular foramen syndrome (JFS)-spectrum disorder. Case Description A 59-year-old woman presented with 4 months of left aural fullness and pulsatile tenderness, positional vertigo, hoarseness, and dysphagia. Examination identified left tongue weakness and nonspecific sensory abnormalities of the ear, pharynx, and throat localizing to cranial nerves IX–XII. Imaging revealed a 3.4 × 1.4 × 2.8 cm lytic lesion extending from the left jugular foramen into the posterior fossa, for which she was referred to neurosurgery and otolaryngology for consideration of resection. A second, much smaller (1.1-cm) lytic lesion in the left posterior occipital bone was incidentally discovered by the surgeon during preoperative consultation. A stereotactic biopsy of the occipital lesion was subsequently recommended, which identified plasma cell neoplasm. Serum studies and skeletal survey were consistent with MM, and a definitive pathologic diagnosis of MM with cranial EP was confirmed by bone marrow biopsy. Conclusion Tumors of the jugular foramen present with a diverse array of lower cranial nerve deficits, including Collet-Sicard syndrome, a rare subset of JFS-spectrum diseases. Paragangliomas are the most common jugular foramen neoplasms, followed by schwannomas and meningiomas; however, many other rare entities have been reported as masqueraders, and diligent work-up with consideration for preliminary biopsy is recommended, particularly in the presence of additional lesions, equivocal imaging findings, or cases arousing high suspicion.

Original languageEnglish (US)
Pages (from-to)386-390
Number of pages5
JournalWorld Neurosurgery
Volume110
DOIs
StatePublished - Feb 1 2018

Fingerprint

Plasmacytoma
Neck
Cranial Nerves
Pharynx
Biopsy
Ear
Plasma Cell Neoplasms
Occipital Bone
Hoarseness
Paraganglioma
Vertigo
Neurilemmoma
Skull Base
Otolaryngology
Neurosurgery
Meningioma
Head and Neck Neoplasms
Deglutition Disorders
Tongue
Referral and Consultation

Keywords

  • Collet-Sicard syndrome
  • Jugular foramen
  • Multiple myeloma
  • Plasmacytoma
  • Skull base

ASJC Scopus subject areas

  • Surgery
  • Clinical Neurology

Cite this

Collet-Sicard Syndrome Attributable to Extramedullary Plasmacytoma of the Jugular Foramen. / Oushy, Soliman; Graffeo, Christopher S.; Perry, Avital; Morris, Jonathan M.; Carlson, Matthew L.; Van Gompel, Jamie.

In: World Neurosurgery, Vol. 110, 01.02.2018, p. 386-390.

Research output: Contribution to journalArticle

Oushy, Soliman ; Graffeo, Christopher S. ; Perry, Avital ; Morris, Jonathan M. ; Carlson, Matthew L. ; Van Gompel, Jamie. / Collet-Sicard Syndrome Attributable to Extramedullary Plasmacytoma of the Jugular Foramen. In: World Neurosurgery. 2018 ; Vol. 110. pp. 386-390.
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abstract = "Background Collet-Sicard syndrome is a rare manifestation of skull base disease involving the jugular and hypoglossal foramina. We report the first case of Collet-Sicard attributable to extramedullary plasmacytoma—multiple myeloma (EP-MM) and the second case of EP-MM precipitating a jugular foramen syndrome (JFS)-spectrum disorder. Case Description A 59-year-old woman presented with 4 months of left aural fullness and pulsatile tenderness, positional vertigo, hoarseness, and dysphagia. Examination identified left tongue weakness and nonspecific sensory abnormalities of the ear, pharynx, and throat localizing to cranial nerves IX–XII. Imaging revealed a 3.4 × 1.4 × 2.8 cm lytic lesion extending from the left jugular foramen into the posterior fossa, for which she was referred to neurosurgery and otolaryngology for consideration of resection. A second, much smaller (1.1-cm) lytic lesion in the left posterior occipital bone was incidentally discovered by the surgeon during preoperative consultation. A stereotactic biopsy of the occipital lesion was subsequently recommended, which identified plasma cell neoplasm. Serum studies and skeletal survey were consistent with MM, and a definitive pathologic diagnosis of MM with cranial EP was confirmed by bone marrow biopsy. Conclusion Tumors of the jugular foramen present with a diverse array of lower cranial nerve deficits, including Collet-Sicard syndrome, a rare subset of JFS-spectrum diseases. Paragangliomas are the most common jugular foramen neoplasms, followed by schwannomas and meningiomas; however, many other rare entities have been reported as masqueraders, and diligent work-up with consideration for preliminary biopsy is recommended, particularly in the presence of additional lesions, equivocal imaging findings, or cases arousing high suspicion.",
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AU - Morris, Jonathan M.

AU - Carlson, Matthew L.

AU - Van Gompel, Jamie

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N2 - Background Collet-Sicard syndrome is a rare manifestation of skull base disease involving the jugular and hypoglossal foramina. We report the first case of Collet-Sicard attributable to extramedullary plasmacytoma—multiple myeloma (EP-MM) and the second case of EP-MM precipitating a jugular foramen syndrome (JFS)-spectrum disorder. Case Description A 59-year-old woman presented with 4 months of left aural fullness and pulsatile tenderness, positional vertigo, hoarseness, and dysphagia. Examination identified left tongue weakness and nonspecific sensory abnormalities of the ear, pharynx, and throat localizing to cranial nerves IX–XII. Imaging revealed a 3.4 × 1.4 × 2.8 cm lytic lesion extending from the left jugular foramen into the posterior fossa, for which she was referred to neurosurgery and otolaryngology for consideration of resection. A second, much smaller (1.1-cm) lytic lesion in the left posterior occipital bone was incidentally discovered by the surgeon during preoperative consultation. A stereotactic biopsy of the occipital lesion was subsequently recommended, which identified plasma cell neoplasm. Serum studies and skeletal survey were consistent with MM, and a definitive pathologic diagnosis of MM with cranial EP was confirmed by bone marrow biopsy. Conclusion Tumors of the jugular foramen present with a diverse array of lower cranial nerve deficits, including Collet-Sicard syndrome, a rare subset of JFS-spectrum diseases. Paragangliomas are the most common jugular foramen neoplasms, followed by schwannomas and meningiomas; however, many other rare entities have been reported as masqueraders, and diligent work-up with consideration for preliminary biopsy is recommended, particularly in the presence of additional lesions, equivocal imaging findings, or cases arousing high suspicion.

AB - Background Collet-Sicard syndrome is a rare manifestation of skull base disease involving the jugular and hypoglossal foramina. We report the first case of Collet-Sicard attributable to extramedullary plasmacytoma—multiple myeloma (EP-MM) and the second case of EP-MM precipitating a jugular foramen syndrome (JFS)-spectrum disorder. Case Description A 59-year-old woman presented with 4 months of left aural fullness and pulsatile tenderness, positional vertigo, hoarseness, and dysphagia. Examination identified left tongue weakness and nonspecific sensory abnormalities of the ear, pharynx, and throat localizing to cranial nerves IX–XII. Imaging revealed a 3.4 × 1.4 × 2.8 cm lytic lesion extending from the left jugular foramen into the posterior fossa, for which she was referred to neurosurgery and otolaryngology for consideration of resection. A second, much smaller (1.1-cm) lytic lesion in the left posterior occipital bone was incidentally discovered by the surgeon during preoperative consultation. A stereotactic biopsy of the occipital lesion was subsequently recommended, which identified plasma cell neoplasm. Serum studies and skeletal survey were consistent with MM, and a definitive pathologic diagnosis of MM with cranial EP was confirmed by bone marrow biopsy. Conclusion Tumors of the jugular foramen present with a diverse array of lower cranial nerve deficits, including Collet-Sicard syndrome, a rare subset of JFS-spectrum diseases. Paragangliomas are the most common jugular foramen neoplasms, followed by schwannomas and meningiomas; however, many other rare entities have been reported as masqueraders, and diligent work-up with consideration for preliminary biopsy is recommended, particularly in the presence of additional lesions, equivocal imaging findings, or cases arousing high suspicion.

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KW - Plasmacytoma

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