CASE REPORTS

INTRAMEDULLARY ENDOMETRIOSIS OF THE CONUS

Amit Agrawal, M.Ch.
Department of Neurosurgery,
K.S. Hegde Medical Academy,
Mangalore, India
Bellore J.P. Shetty, M.S.
Department of Orthopedics,
K.S. Hegde Medical Academy,
Mangalore, India
Jagadeesh H. Makannavar, M.D. rhage. The patient underwent an emergency D12–L2 laminectomy and microdecom-
Department of Pathology,
K.S. Hegde Medical Academy,
Mangalore, India
Lathika Shetty, M.D.
Department of Radiology,
K.S. Hegde Medical Academy,
Mangalore, India
MEDULLARIS: CASE REPORT
OBJECTIVE: Intraspinal endometriosis is an extremely rare condition with character-
istic symptoms, including lower back pain that increases in severity during each
menstrual cycle.
METHODS: Here, we report a case of endometriosis involving the conus cauda region.
This patient presented with acute deterioration secondary to hemorrhage. We also
review the relevant literature.
RESULTS: Magnetic resonance imaging scans of the dorsolumbar region showed a
mass lesion within the spinal canal at the L1–L2 level with evidence of acute hemor-
pression of the lesion. The histological and immunohistochemical features were
characteristic of intraspinal endometriosis.
CONCLUSION: Intraspinal endometriosis must be recognized as a potential cause of
periodic neurological signs and symptoms in young and middle-aged women.
KEY WORDS: Conus medullaris, Endometriosis, Spinal canal, Spinal cord diseases
Neurosurgery 59:428, 2006 DOI: 10.1227/01.NEU.0000223375.23617.DC www.neurosurgery-online.com

Jayaprakash Shetty, M.D.

Department of Pathology,
K.S. Hegde Medical Academy,
Mangalore, India
Vikram Shetty, M.S.
Department of Orthopedics,
K.S. Hegde Medical Academy,
Mangalore, India
Reprint requests:
Amit Agrawal, M.Ch.,
Department of Neurosurgery,
K.S. Hegde Medical Academy,
Deralakatte, Mangalore,
Karnataka 575018, India
Received, August 26, 2005.
Accepted, March 27, 2006.
E
ndometriosis (EM) is defined by the
presence of tissue that is histologically
and functionally similar to the endome-
trium outside the uterus. EM has been mostly
reported in the pelvis. Intraspinal EM (IEM) is
extremely rare, and there are only six case
reports in the literature (1–6). In the present
article, we discuss the clinical aspects, radio-
logical features, and surgical treatment of IEM
and review the relevant literature.
CASE REPORT
A 40-year-old woman presented with a 12-
year history of progressive pain in the lumbosa-
cral region radiating to both lower limbs, which
was correlated with her menstrual period. At
the time of presentation, she reported worsen-
ing of these symptoms accompanied by weak-
ness in lower limbs (Grade 4/5), constipation,
and urinary hesitancy for 2 days at the onset of
the menstrual cycle. While awaiting investiga-
tion, she suddenly developed urinary retention
and weakness of both lower limbs, with sensory
loss below the L1 level. General and systemic
examinations were unremarkable. There was no
tenderness or deformity of the dorsolumbar
spine. The patient had hypotonia in both lower
limbs, with Grade 0/5 power in the left lower
limb and Grade 3/5 power in the right lower
limb, along with sensory loss below the L1 level.
Ankle and knee jerks were absent on the left
side and sluggish on the right side. Blood inves-
tigations were normal. An x-ray of the dor-
solumbar spine was normal. Magnetic reso-
nance imaging (MRI) scans of the dorsolumbar
region showed an isointense lesion on T1-
weighted images (Fig. 1), with a thin, peripheral
hypointense ring within the spinal canal at the
L1–L2 level. The lesion was hypointense on T2-
weighted images (Fig. 1), with T2 shortening at
the periphery suggestive of acute hemorrhage.
Although our differential diagnosis was of a
tumor with bleeding, we could not perform an
angiogram because of a lack of facilities and
financial constraints. The patient underwent an
emergency D12–L2 laminectomy and microde-
compression of the lesion. During surgery, after
opening the dura, xanthochromic cerebrospinal
fluid leaked out. A mass was identified in the
spinal canal involving the conus cauda region,
which was densely adhesive with the conus and
cauda equine and was not separable from the
cord tissue and nerve roots. The mass consisted
of firm, fleshy, and relatively avascular tissue,
with necrosis and old hemorrhage. The tumor

E428 | VOLUME 59 | NUMBER 2 | AUGUST 2006 www.neurosurgery-online.com

 INTRAMEDULLARY ENDOMETRIOSIS
FIGURE 1. T1-weighted sagittal MRI scan (A) showing an isointense in-
traspinal lesion involving the conus cauda region. On a T2-weighted sagittal
MRI scan (B), the lesion is becoming hypointense with a rim of T2 shortening.
tissue was partially removed without further injury to the nerve
roots, and hemostasis was achieved without difficulty. Histolog-
ical examination of the resected tissue revealed islands of endo-
metrial glands lined by tall, columnar epithelium. The glands
were surrounded by stromal cells irregularly infiltrating into the
surrounding mesenchyme (Fig. 2A). Some of the glands were
dilated and had myxoid secretions. Paraffin sections were sub-
jected to standard indirect immunoperoxidase staining using
monoclonal antibodies to estrogen and progesterone receptors
and 3131-diaminobenzidine tetrahydrochloride-H2O2 as a chro-
mogen. Sections were counterstained with Harris’ hematoxylin.
Indirect immunoperoxidase staining using antibodies to estrogen
and progesterone receptors revealed strong labeling of both
glands and stroma for progesterone receptors (Fig. 2B); the glan-
dular labeling was relatively weak, whereas the stromal cells
were stained strongly by antibodies to estrogen receptors (Fig.
2C). The histological and immunohistochemical features were
characteristic of IEM. On gynecological examination, the vulva
and vagina were normal, the uterus was smooth, and its position,
activity, and size were normal. No abnormal lumps were palpa-
ble in the regions of the bilateral appendages. Ultrasound exam-
ination of the abdomen was normal. After surgery, there was
improvement in motor power in the left lower limb to Grade 3/5
and in the right lower limb to Grade 4/5. The patient’s radicular
pain was relieved, and she was on self-intermittent catheteriza-
tion for bladder dysfunction. The patient was started on danazol
(400 mg daily) for pain relief. She also underwent bilateral oo-
phorectomy, and no evidence of pelvic EM was detected during
surgery. At the 3-month follow-up examination, the patient
could walk without support and had regained bowel and blad-
der control. Follow-up MRI scans showed regression of the le-
sion (Fig. 3).
NEUROSURGERY
OF CONUS MEDULLARIS
FIGURE 2. A, histological section showing endometrial glands with sur-
rounding stroma entrapped in the mesenchymal tissue. Progesterone recep-
tors (B) are expressed both in endometrial glands and stromal cells,
whereas expression of estrogen receptors more evident in stromal cells (C).
A, hematoxylin-eosin; original magnification, ⫻240. B, immunoperoxidase
stain for progesterone receptors; original magnification, ⫻240. C, immu-
noperoxidase stain for estrogen receptors; original magnification, ⫻240.
DISCUSSION
The major clinical characteristics of IEM are a series of
symptoms associated with the menstrual period, including
meningeal irritation syndromes or symptoms deriving from
compression of the nidus to nerve tissue of the spinal canal,
such as pain in the lumbar region or lower extremities, dys-
esthesia of the bladder or rectum, and even paraplegia (5).
General physical and gynecological examinations are always
normal among patients with IEM (2, 5). Our patient also
presented with classical symptoms and normal physical and
gynecological examination. There have been many hypotheses
on the causes of IEM. However, in terms of anatomy, IEM
probably results from the reverse transport of endometriotic
tissue via the Betson’s venous plexus (5). Cerebrospinal fluid
studies show normal pressure, xanthochromia, and abundant
old erythrocytes, along with normal white blood cells under
the microscope, moderate protein levels, and normal glucose
levels. X-rays, computed tomographic scans, and MRI scans
cannot diagnose IEM, except for showing a tumorous occu-
pancy in the spinal canal, making it difficult to distinguish
from tumors. Tumor-associated antigen (CA125) and endome-
trium antibodies might be positive on immunostaining (2, 5).
In the present case, MRI scans were suggestive of a tumorous
lesion with bleed. However, the exact nature of the lesion
could only be confirmed after histopathological examination
and was further supported by immunostaining. Similar to
other types of EM, IEM is a hormone-dependent disease, and
there are few published reports on treating spinal cord EM (1,
2, 5). Drug therapy is recommended if there is sufficient pre-
operative clinical evidence to suspect IEM (2). Surgery should
be considered for patients in whom occupancy of the nidus in
the spinal canal might cause spinal cord or cauda equine
injury, in patients with severe symptoms that are not relieved
VOLUME 59 | NUMBER 2 | AUGUST 2006 | E428
AGRAWAL ET AL.
FIGURE 3. Follow-up T1- (A) and T2-weighted (B) MRI scans showing
regression in the size of the lesion (compare with Fig. 1).
by drugs, and in patients with frequent recurrence. During
surgery, as much of the endometrioid tissue as possible
should be removed while protecting the nerve tissue (2, 5).
Total excision of the endometriotic lesion, followed by a bilat-
eral oophorectomy and aromatase inhibitor therapy, will lead
to clinical improvement (3). Drug therapy is also necessary
after surgery, especially in cases without radical surgery, as in
the present case, to prevent recurrence (2, 3, 5). In addition,
attempts at the total removal of the spinal cord EM might be
safer after achieving pharmacological control (3). IEM must be
recognized as a potential cause of periodic neurological signs
and symptoms in young and middle-aged women because
timely intervention and appropriate management in patients
with neurological symptoms will result in control of the dis-
ease and an improvement in neurological functions.
REFERENCES
1. Carta F, Guiducci G, Fulcheri E, Bernucci C, Rivano C: Radicular compression
by extradural spinal endometriosis. Case report. Acta Neurochir (Wien)
114:68–71, 1992.
2. Duke R, Fawcett P, Booth J: Recurrent subarachnoid hemorrhage due to
endometriosis. Neurology 45:1000–1002, 1995.
3. Erbayraktar S, Acar B, Saygili U, Kargi A, Acar U: Management of intramed-
ullary endometriosis of the conus medullaris. A case report. J Reprod Med
47:955–958, 2002.
E428 | VOLUME 59 | NUMBER 2 | AUGUST 2006
4. Richer K: Endometrioid carcinoma of the spinal canal [in German].
Geburtshilfe Frawenheilk 37:771–775, 2002.
5. Sun Z, Wang Y, Zhao L, Ma L: Intraspinal endometriosis: A case report. Chin
Med J (Engl) 115:622–623, 2002.
6. Thibodeau LL, Prioleau GR, Manuelidis EE, Merino MJ, Heafner MD: Cere-
bral endometriosis: Case report. J Neurosurg 66:609–610, 1987.
Acknowledgments
We thank Dr. S.K. Shankar, Professor and Head, Department of Neuropathol-
ogy, National Institute of Mental Health and Neurosciences, Bangalore, for his
help with the immunostaining of biopsy material for this case report. We also
acknowledge, with thanks, the financial assistance provided by Mr. Vinay
Hegde, President, NITTE Education Trust, to complete this study. None of the
authors have received additional funding from any commercial companies.
COMMENTS
A grawal et al. present a rare case of endometriosis involving the
conus medullaris and presenting with an acute neurological de-
terioration secondary to hemorrhage. As this case demonstrates, in-
termittent symptoms fluctuating with a women’s menstrual cycle
should raise suspicion for this rare clinical entity. Although a specific
management strategy is difficult to define, in this case, the authors
demonstrated sound clinical judgment, exemplified by the patient’s
significant neurological recovery. In the absence of an acute neuro-
logical deterioration, it would seem that a trial of medical manage-
ment is not an unreasonable option.
Michael G. Kaiser
New York, New York
T he authors describe a rare but interesting case of intraspinal en-
dometriosis. The case is well-documented with radiological, his-
tological, and immunohistochemical studies. It is interesting that no
evidence of endometriosis was identified elsewhere in this patient,
indicating that presurgical diagnosis of this entity may be challenging.
However, awareness of this entity in the differential diagnosis of signs
and symptoms referable to a mass lesion in the lumbosacral cord in
young women is important because presurgical medical therapy has
theoretical advantages for diagnostic and therapeutic purposes.
Ken Aldape
Neuropathologist
Houston, Texas
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