Nuchal Fibrocartilaginous Pseudotumor in a

10-Year-Old Girl
Eduardo Luévano-Flores, MD; Arturo Aguirre-Madrid, MD

● We report a case of a 10-year-old girl who, 4 years after
a trauma to the neck, developed a soft tissue mass in the
midline at the C5-6 level. The lesion was not attached to
the bone and was composed of fibrocartilaginous tissue,
similar to the nuchal fibrocartilaginous pseudotumor, a
rare, recently reported condition that has been described
in the medical literature in only 4 cases, all of them adults.
(Arch Pathol Lab Med. 2000;124:1217–1219)
out calcifications (Figure 1). The lesion was resected, and 1 year
later, the patient remains well and has no evidence of recurrence.
The excised nodule consisted of fibrocartilaginous tissue sur-
rounded by dense connective tissue of the nuchal ligament (Fig-
ure 2). The central area showed a fibrocartilaginous matrix, with
chondrocytes that were small and lacked nuclear enlargement or
nucleoli (Figure 3). Neither mitotic figures nor binucleated chon-
drocytes were found. Under polarized light, the extracellular ma-
trix contained abundant polarizing collagen in a somewhat dis-
organized fashion; the elastic tissue stain showed scarce frag-
mented elastic fibers in the center of the nodule. At the periphery,

R ecently, O’Conell et al1 described a fibrocartilaginous

nodule in the posterior aspect of the base of the neck,
at the junction of the nuchal ligament and the deep cer-
abundant elastic fibers were apparent. There were no calcifica-
tions, hemosiderin deposits, or inflammatory infiltrate.

vical fascia, that simulated a tumor. They found this lesion COMMENT
in 3 patients with a history of trauma in motor vehicle In the soft tissues, many nonneoplastic conditions can
crashes; in their opinion, the masses probably arose produce the clinical appearance of a mass; some of them
through a process of fibrocartilaginous metaplasia in pre- are secondary to inflammatory processes, trauma, or for-
viously traumatized and degenerated nuchal ligament tis- eign material, and others correspond to amyloid deposi-
sue. Therefore, they called this lesion nuchal fibrocartilagi- tion or heterotopic distrophic calcification. Still others are
nous pseudotumor. Two of their patients were women, ages due to proliferating fibrous tissue, which can be confused
37 and 40 years, and 1 was a 53-year-old man; they all with true neoplastic conditions.
had sustained previous neck injuries in automobile crash-
es, 27 and 20 years earlier in the women and 2 months
earlier in the man. In their review of the literature, the
authors found only a single report similar in all aspects
to their cases. The report, by Lewinnek and Peterson,2 de-
scribed a 39-year-old woman with no history of trauma
who had a fibrocartilaginous mass in the nuchal ligament.
We report the case of an identical lesion in a 9-year-old
girl with a history of trauma to the neck incurred in an
automobile crash 4 years earlier.

REPORT OF A CASE
The patient was a 10-year-old girl who was hit by a car 4 years
earlier. She presented with pain and discomfort in the posterior
aspect of the neck and required an orthopedic collar for 3 weeks.
Subsequently, the complaints disappeared. Four years later, she
developed a 2.5-cm, firm nodule that appeared at the C5-6 level
and that was painless and not attached to the bone. Linear to-
mography of the neck showed no detectable abnormalities; how-
ever, a computed tomographic scan of the neck showed a 1.5-cm
nodular thickening of the nuchal ligament at the C5-6 level, with-

Accepted for publication December 1, 1999.

From the Departments of Pathology (Dr Luévano-Flores) and Ortho-
pedic Surgery (Dr Aguirre-Madrid), Hospital Clı́nica del Parque, Chi-
huahua, Chihuahua, México.
Reprints: Eduardo Luévano-Flores, MD, Morelos 1407-B, Colonia Figure 1. A computed tomographic scan of the cervical area showed
Centro c.p. 31000, Chihuahua, Chihuahua, México (e-mail: luevano@ a nodular thickening at the tip of the spinous process of the C5 ver-
infosel.net.mx). tebrae (arrow).
Arch Pathol Lab Med—Vol 124, August 2000 Nuchal Fibrocartilaginous Pseudotumor—Luévano-Flores & Aguirre-Madrid 1217
Figure 2. Edge of the lesion showing fibrocartilaginous tissue surrounded by disorganized collagen bundles of the fascia; there is no inflammatory
infiltrate or calcification (hematoxylin-eosin, original magnification 340).
Figure 3. Central area of the lesion showing cellular fibrocartilage. Note the cracks within the collagen-rich matrix (hematoxylin-eosin, original
magnification 3100).

The nuchal fibrocartilaginous pseudotumor described
by O’Conell et al is a lesion with a characteristically spe-
cific location in the posterior aspect of the nuchal ligament,
at its attachment to the deep cervical fascia, in the midline
at the level of C4-5 or C5-6. Histologically, the lesion con-
sists of a poorly delineated, moderately cellular fibrocar-
tilaginous nodule, with cracks within the collagen-rich
matrix. The chondrocytes within the mass are small and
lack nuclear enlargement or nucleoli. Mitotic figures and
binucleated chondrocytes are absent. At the interface of
the nuchal ligament with the fibrocartilaginous mass, the
ligamentous tissue appears degenerated, with fragmen-
tation of the elastic fibers. The adjacent paraspinal muscles
and overlying superficial fascia are histologically normal,
without interstitial scarring or hemosiderin deposits.
A lesion that can occur in this area is a so-called nuchal
fibroma.3 However, this lesion is not confined to the mid-
line, lacks an association with ligaments, and occurs su-
perficial to the fascia. The histologic picture corresponds
to sheets of hypocellular dense collagen, with interspersed
mature fat, small vessels, and entrapped nerve fibers;
1218 Arch Pathol Lab Med—Vol 124, August 2000
since it is not composed of fibrocartilage, it resembles
more of a fibrolipoma, lipomatosis, scar, or elastofibroma.
Other entities that show cartilaginous tissue, which are
not characteristically located in the cervical region but
which should be taken in to account for the differential
diagnosis, are as follows. The soft tissue chondroma4 pre-
sents with well-defined lobules of hyaline cartilage, usu-
ally in association with a tendon or a tendon sheath and
most commonly in the distal extremities. The synovial
chondromatosis5 may occasionally present as a soft tissue
mass in relationship to a synovial-lined joint and is char-
acterized by lobules of hyaline cartilage with an overlying
synovial membrane. The mesenchymal chondrosarcoma6
shows an obviously malignant undifferentiated compo-
nent with small blue cells, in addition to areas of low-
grade hyaline cartilage. The extraskeletal myxoid chondro-
sarcoma is also a malignant deep soft tissue mass, in
which fibrocartilage is not a feature. The calcifying apo-
neurotic fibroma7 has a predilection for the distal extrem-
ities; the cartilaginous component is microscopic and em-
bedded within a fibromatosis-like background. Myositis
Nuchal Fibrocartilaginous Pseudotumor—Luévano-Flores & Aguirre-Madrid
ossificans may have fibrocartilage but is also accompanied
by a reactive spindle cell component, with woven bone in
various stages of maturation.8 Other entities, such as the
calcifying fibrous pseudotumor and the fibromatosis, do
not have a fibrocartilaginous component.9
Crockard et al10 described 5 elderly patients with space-
occupying lesions, within the anterior aspect of upper spi-
nal canal, posterior to the odontoid, resembling degener-
ating intervertebral disk, which compressed the upper spi-
nal cord. However, the localization was not in the nuchal
area. In addition, pathologically the masses were com-
posed of a mixture of degenerated ligament tissue, fibro-
cartilage, necrotic material, and fibrin, with calcification
and vascular ingrowth. The authors speculated that the
masses probably arose from an exuberant repair reaction
after partial tears of the transverse ligament of the odon-
toid process.
The nuchal ligament is an anatomic structure made of
a fibroelastic membrane that extends from the occipital
bone to the spine of the seventh cervical vertebra. Scapi-
nelli11 described in this ligament the formation of sesa-
moid bones, developing in connective tissue with a pre-
vious stage of fibrocartilaginous metaplasia. He consid-
ered that this change is secondary to mechanical factors,
acting during the neck flexion, when the thick nuchal lig-
ament is subjected to pressure and trauma as it passes
over the spinous processes of the cervical vertebrae.
O’Connell et al considered that trauma at the C4-5 or
C5-6 level, which is more susceptible to hyperflexion in-
juries during deceleration events such as automobile crash-
Arch Pathol Lab Med—Vol 124, August 2000 Nuchal Fibrocartilaginous Pseudotumor—Luévano-Flores & Aguirre-Madrid 1219
es, generates a process of fibrocartilaginous metaplasia,
which in turn produces a pseudotumoral mass in this par-
ticular site. The range of time between the trauma and the
onset of the lesion is variable, from 27 years to 2 months.
The case described by Lewinnek and Peterson2 had no
history of trauma, all the previous reported cases have
been in adults, and we did not find any report of this
condition in children. The clinicopathologic findings in
our pediatric case were similar to the ones described in
the nuchal fibrocartilaginous pseudotumor.
References
1. O’Conell JX, Janzen DL, Hughes TR. Nuchal fibrocartilaginous pseudotu-
mor, a distinctive soft-tissue lesion associated with prior neck injury. Am J Surg
Pathol. 1997;21:836–840.
2. Lewinnek GE, Peterson SE. A calcified fibrocartilaginous nodule in the li-
gamentum nuchae presenting as a tumor. Clin Orthop. 1978;136:163–165.
3. Balachandran K, Allen PW, MacCormac LB. Nuchal fibroma: a clinicopath-
ological study of nine cases. Am J Surg Pathol. 1995;19:313–317.
4. Chung EB, Enzinger FM. Chondroma of soft parts. Cancer. 1978;41:1414–
1424.
5. Sviland L, Malcolm AJ. Synovial chondromatosis presenting as painless soft
tissue mass: a report of 19 cases. Histopathology. 1995;27:275–279.
6. Nakashima Y, Unni KK, Shaves TC, Swee RG, Dahlin DC. Mesenchymal
chondrosarcoma of bone and soft tissue: a review of 11 cases. Cancer. 1986;57:
2444–2453.
7. Allen PW, Enzinger FM. Juvenile aponeurotic fibroma. Cancer. 1970;26:
857–867.
8. Enzinger FM, Weiss SW. Osseous tumors of soft tissue. In: Soft Tissue Tu-
mors. 3rd ed. St Louis, Mo: CV Mosby; 1995:1013–1021.
9. Fetsch JE, Montgomery EA, Meis JM. Calcifying fibrous pseudotumor. Am J
Surg Pathol. 1993;17:502–508.
10. Crockard HA, Sett P, Geddes JF, Stevens JM, Kendall BE, Pringle JAS. Dam-
aged ligaments at the craniocervical junction presenting as an extradural tumour:
a differential diagnosis in the elderly. J Neurol Neurosurg Psychiatry. 1991;54:
817–821.
11. Scapinelli R. Sesamoid bones in the ligamentum nuchae of man. J Anat.
1963;97:417–422.