A rare case of metastatic extramammary Paget disease of the spine and review of the literature

A. Karim Ahmed, C. Rory Goodwin, Nancy Abu-Bonsrah, Doreen Nguyen, Daniel M. Sciubba

Research output: Contribution to journalArticle

Abstract

Extramammary Paget disease is an intraepithelial neoplasm affecting cells rich in apocrine glands-often located in the vulvar, scrotal, or perianal region. It typically affects older patients, between the ages of 50 and 80. years old, and is most often limited to the epidermis. A 47-year-old Asian male first presented with enlargement of the right inguinal lymph node. A subsequent biopsy revealed extrammamary Paget disease of the scrotum. The patient eventually developed significant worsening back pain with bilateral lower extremity numbness and weakness three months later. Imaging demonstrated a pathologic compression fracture of the L4 vertebral body with metastatic epidural spinal cord compression. The patient underwent surgical decompression of the spine with bilateral L4 laminectomy, resection of epidural tumor, and pedicle screw fixation from L2 to S1. Surgical pathology demonstrated metastatic adenocarcinoma consistent with extramammary Paget disease. Although two other case reports have described spinal metastases from extramammary Paget disease, to the author's knowledge, this represents the first report of surgical decompression and fusion for extramammary Paget disease of the spine.

LanguageEnglish (US)
JournalJournal of Clinical Neuroscience
DOIs
StateAccepted/In press - 2017

Fingerprint

Extramammary Paget's Disease
Spine
Surgical Decompression
Epidural Neoplasms
Apocrine Glands
Compression Fractures
Surgical Pathology
Spontaneous Fractures
Spinal Cord Compression
Scrotum
Laminectomy
Hypesthesia
Groin
Carcinoma in Situ
Back Pain
Epidermis
Lower Extremity
Adenocarcinoma
Lymph Nodes
Neoplasm Metastasis

Keywords

  • Extramammary
  • Hemolytic anemia
  • Metastasis
  • Paget disease
  • Spine
  • Surgery

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology
  • Physiology (medical)

Cite this

A rare case of metastatic extramammary Paget disease of the spine and review of the literature. / Ahmed, A. Karim; Goodwin, C. Rory; Abu-Bonsrah, Nancy; Nguyen, Doreen; Sciubba, Daniel M.

In: Journal of Clinical Neuroscience, 2017.

Research output: Contribution to journalArticle

@article{aa5ee20412234a769a6854e2d972c6d1,
title = "A rare case of metastatic extramammary Paget disease of the spine and review of the literature",
abstract = "Extramammary Paget disease is an intraepithelial neoplasm affecting cells rich in apocrine glands-often located in the vulvar, scrotal, or perianal region. It typically affects older patients, between the ages of 50 and 80. years old, and is most often limited to the epidermis. A 47-year-old Asian male first presented with enlargement of the right inguinal lymph node. A subsequent biopsy revealed extrammamary Paget disease of the scrotum. The patient eventually developed significant worsening back pain with bilateral lower extremity numbness and weakness three months later. Imaging demonstrated a pathologic compression fracture of the L4 vertebral body with metastatic epidural spinal cord compression. The patient underwent surgical decompression of the spine with bilateral L4 laminectomy, resection of epidural tumor, and pedicle screw fixation from L2 to S1. Surgical pathology demonstrated metastatic adenocarcinoma consistent with extramammary Paget disease. Although two other case reports have described spinal metastases from extramammary Paget disease, to the author\{textquoteleft}s knowledge, this represents the first report of surgical decompression and fusion for extramammary Paget disease of the spine.",
keywords = "Extramammary, Hemolytic anemia, Metastasis, Paget disease, Spine, Surgery",
author = "Ahmed, {A. Karim} and Goodwin, {C. Rory} and Nancy Abu-Bonsrah and Doreen Nguyen and Sciubba, {Daniel M.}",
year = "2017",
doi = "10.1016/j.jocn.2017.08.018",
language = "English (US)",
journal = "Journal of Clinical Neuroscience",
issn = "0967-5868",
publisher = "Churchill Livingstone",

}

TY - JOUR

T1 - A rare case of metastatic extramammary Paget disease of the spine and review of the literature

AU - Ahmed,A. Karim

AU - Goodwin,C. Rory

AU - Abu-Bonsrah,Nancy

AU - Nguyen,Doreen

AU - Sciubba,Daniel M.

PY - 2017

Y1 - 2017

N2 - Extramammary Paget disease is an intraepithelial neoplasm affecting cells rich in apocrine glands-often located in the vulvar, scrotal, or perianal region. It typically affects older patients, between the ages of 50 and 80. years old, and is most often limited to the epidermis. A 47-year-old Asian male first presented with enlargement of the right inguinal lymph node. A subsequent biopsy revealed extrammamary Paget disease of the scrotum. The patient eventually developed significant worsening back pain with bilateral lower extremity numbness and weakness three months later. Imaging demonstrated a pathologic compression fracture of the L4 vertebral body with metastatic epidural spinal cord compression. The patient underwent surgical decompression of the spine with bilateral L4 laminectomy, resection of epidural tumor, and pedicle screw fixation from L2 to S1. Surgical pathology demonstrated metastatic adenocarcinoma consistent with extramammary Paget disease. Although two other case reports have described spinal metastases from extramammary Paget disease, to the author's knowledge, this represents the first report of surgical decompression and fusion for extramammary Paget disease of the spine.

AB - Extramammary Paget disease is an intraepithelial neoplasm affecting cells rich in apocrine glands-often located in the vulvar, scrotal, or perianal region. It typically affects older patients, between the ages of 50 and 80. years old, and is most often limited to the epidermis. A 47-year-old Asian male first presented with enlargement of the right inguinal lymph node. A subsequent biopsy revealed extrammamary Paget disease of the scrotum. The patient eventually developed significant worsening back pain with bilateral lower extremity numbness and weakness three months later. Imaging demonstrated a pathologic compression fracture of the L4 vertebral body with metastatic epidural spinal cord compression. The patient underwent surgical decompression of the spine with bilateral L4 laminectomy, resection of epidural tumor, and pedicle screw fixation from L2 to S1. Surgical pathology demonstrated metastatic adenocarcinoma consistent with extramammary Paget disease. Although two other case reports have described spinal metastases from extramammary Paget disease, to the author's knowledge, this represents the first report of surgical decompression and fusion for extramammary Paget disease of the spine.

KW - Extramammary

KW - Hemolytic anemia

KW - Metastasis

KW - Paget disease

KW - Spine

KW - Surgery

UR - http://www.scopus.com/inward/record.url?scp=85028763451&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85028763451&partnerID=8YFLogxK

U2 - 10.1016/j.jocn.2017.08.018

DO - 10.1016/j.jocn.2017.08.018

M3 - Article

JO - Journal of Clinical Neuroscience

T2 - Journal of Clinical Neuroscience

JF - Journal of Clinical Neuroscience

SN - 0967-5868

ER -