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Nuchal Fibrocartilaginous Pseudotumor in a 10-Year Old Girl.



Recently, O'Conell et al[1] described a fibrocartilaginous nodule in the posterior aspect of the base of the neck, at the junction of the nuchal ligament and the deep cervical fascia The deep cervical fascia (or fascia colli in older texts) lies under cover of the Platysma, and invests the neck; it also forms sheaths for the carotid vessels, and for the structures situated in front of the vertebral column. , that simulated a tumor. They found this lesion in 3 patients with a history of trauma in motor vehicle crashes; in their opinion, the masses probably arose through a process of fibrocartilaginous metaplasia in previously traumatized and degenerated nuchal ligament tissue. Therefore, they called this lesion nuchal nuchal (nyōōˑ·kl),
adj pertaining to the posterior or nape of the neck.
 fibrocartilaginous pseudotumor. Two of their patients were women, ages 37 and 40 years, and 1 was a 53-year-old man; they all had sustained previous neck injuries in automobile crashes, 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] described 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 tomography of the neck showed no detectable abnormalities; however, a computed tomographic scan of the neck showed a 1.5-cm nodular thickening of the nuchal ligament at the C5-6 level, without calcifications (Figure 1). The lesion was resected, and 1 year later, the patient remains well and has no evidence of recurrence.

[Figure 1 ILLUSTRATION OMITTED]

The excised nodule consisted of fibrocartilaginous tissue surrounded by dense connective tissue of the nuchal ligament (Figure 2). The central area showed a fibrocartilaginous matrix, with chondrocytes that were small and lacked nuclear enlargement or nucleoli nucleoli

plural form of nucleolus.
 (Figure 3). Neither mitotic figures nor binucleated bi·nu·cle·ate   also bi·nu·cle·at·ed or bi·nu·cle·ar
adj.
Having two nuclei.

Adj. 1. binucleated - having two nuclei
binuclear, binucleate
 chondrocytes were found. Under polarized light, the extracellular matrix contained abundant polarizing collagen in a somewhat disorganized dis·or·gan·ize  
tr.v. dis·or·gan·ized, dis·or·gan·iz·ing, dis·or·gan·iz·es
To destroy the organization, systematic arrangement, or unity of.
 fashion; the elastic tissue stain showed scarce fragmented elastic fibers in the center of the nodule. At the periphery, abundant elastic fibers were apparent. There were no calcifications, hemosiderin hemosiderin /he·mo·sid·er·in/ (he?mo-sid´er-in) an insoluble form of tissue storage iron, visible microscopically both with and without the use of special stains.

he·mo·sid·er·in
n.
 deposits, or inflammatory infiltrate.

[Figure 2-3 ILLUSTRATION OMITTED]

COMMENT

In the soft tissues, many nonneoplastic conditions can produce the clinical appearance of a mass; some of them are secondary to inflammatory processes, trauma, or foreign material and others correspond to amyloid deposition or heterotopic heterotopic

pertaining to heterotopia.
 distrophic calcification. Still others are due to proliferating fibrous tissue, which can be confused with true neoplastic neoplastic /neo·plas·tic/ (ne?o-plas´tik)
1. pertaining to a neoplasm.

2. pertaining to neoplasia.


neoplastic

pertaining to neoplasia or a neoplasm.
 conditions.

The nuchal fibrocartilaginous pseudotumor described by O'Conell et al is a lesion with a characteristically specific 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 consists of a poorly delineated, moderately cellular fibrocartilaginous 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 fragmentation of the elastic fibers. The adjacent paraspinal muscles and overlying overlying

suffocation of piglets by the sow. The piglets may be weak from illness or malnutrition, the sow may be clumsy or ill, the pen may be inadequate in size or poorly designed so that piglets cannot escape.
 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 fibroma /fi·bro·ma/ (fi-bro´mah) pl. fibromas, fibro´mata   a tumor composed mainly of fibrous or fully developed connective tissue. .[3] However, this lesion is not confined to the midline, lacks an association with ligaments, and occurs superficial to the fascia. The histologic picture corresponds to sheets of hypocellular dense collagen, with interspersed mature fat, small vessels, and entrapped nerve fibers; since it is not composed of fibrocartilage fibrocartilage /fi·bro·car·ti·lage/ (-kahr´ti-laj) cartilage of parallel, thick, compact collagenous bundles, separated by narrow clefts containing the typical cartilage cells (chondrocytes). , it resembles more of a fibrolipoma, lipomatosis, scar, or elastofibroma.

Other entities that show cartilaginous cartilaginous /car·ti·lag·i·nous/ (kahr?ti-laj´i-nus) consisting of or of the nature of cartilage.

car·ti·lag·i·nous
adj.
1. Chondral.

2.
 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 chondroma chondroma /chon·dro·ma/ (kon-dro´mah) pl. chondromas, chondro´mata   a benign tumor or tumor-like growth of mature hyaline cartilage. [4] presents with well-defined lobules Lobules
A small lobe or subdivision of a lobe (often on a gland) that may be seen on the surface of the gland by bumps or bulges.

Mentioned in: Fibrocystic Condition of the Breast
 of hyaline cartilage, usually in association with a tendon or a tendon sheath and most commonly in the distal extremities. The synovial chondromatosis[5] may occasionally present as a soft tissue mass in relationship to a synovial-lined joint and is characterized by lobules of hyaline cartilage with an overlying synovial membrane. The mesenchymal chondrosarcoma[6] shows an obviously malignant undifferentiated component with small blue cells, in addition to areas of low-grade hyaline cartilage. The extraskeletal myxoid myxoid /myx·oid/ (mik´soid) mucoid.

myx·oid
adj.
Containing or resembling mucus; mucoid.



myxoid

resembling mucus.

myxoid adjective 1.
 chondrosarcoma is also a malignant deep soft tissue mass, in which fibrocartilage is not a feature. The calcifying calcifying

mineralized.


calcifying aponeurotic fibroma
locally aggressive nodular masses that involve membranous bones, particularly those of the canine skull (zygomatic arch), and rarely metastasize.
 aponeurotic fibroma[7] has a predilection for the distal extremities; the cartilaginous component is microscopic and embedded within a fibromatosis-like background. Myositis 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 al[10] described 5 elderly patients with space-occupying lesions, within the anterior aspect of upper spinal canal, posterior to the odontoid odontoid /odon·toid/ (o-don´toid) like a tooth.

o·don·toid
adj.
1. Shaped like a tooth.

2. Of or relating to the odontoid process.
, resembling degenerating intervertebral disk, which compressed the upper spinal cord. However, the localization was not in the nuchal area. In addition, pathologically the masses were composed of a mixture of degenerated ligament tissue, fibrocartilage, 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 odontoid process odontoid process
n.
A small, toothlike, upward projection from the second vertebra of the neck around which the first vertebra rotates.


odontoid process (ōdon´toid),
.

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. Scapinelli[11] described in this ligament the formation of sesamoid bones (Anat.) small bones or cartilages formed in tendons, like the patella and pisiform in man.

See also: Sesamoid
, developing in connective tissue with a previous stage of fibrocartilaginous metaplasia. He considered that this change is secondary to mechanical factors, acting during the neck flexion, when the thick nuchal ligament 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 injuries during deceleration events such as automobile crashes, generates a process of fibrocartilaginous metaplasia, which in turn produces a pseudotumoral mass in this particular

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 Peterson[2] 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 pediatric /pe·di·at·ric/ (pe?de-at´rik) pertaining to the health of children.

pe·di·at·ric
adj.
Of or relating to pediatrics.
 case were similar to the ones described in the nuchal fibrocartilaginous pseudotumor.

References

[1.] O'Conell JX, Janzen DL, Hughes TR. Nuchal fibrocartilaginous pseudotumor, 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 Calcified
Hardened by calcium deposits.

Mentioned in: Heart Valve Repair
 fibrocartilaginous nodule in the ligamentum nuchae presenting as a tumor. Clin Orthop. 1978;136:163-165.

[3.] Balachandran K, Allen PW, MacCormac LB. Nuchal fibroma: a clinicopathological study of nine cases. Am J Surg Patrol. 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 osseous /os·se·ous/ (os´e-us) of the nature or quality of bone; bony.

os·se·ous
adj.
Composed of, containing, or resembling bone; bony.
 tumors of soft tissue. In: Soft Tissue Tumors. 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. Damaged ligaments at the craniocervical junction presenting as an extradural extradural

situated or occurring outside the dura mater. See also epidural.
 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.

Accepted for publication December 1, 1999.

From the Departments of Pathology (Dr Luevano-Flores) and Orthopedic Surgery (Dr Aguirre-Madrid), Hospital Clinica del Parque, Chihuahua, Mexico.

Reprints: Eduardo Luevano-Flores, MD, Morelos 1407-B, Colonia Centro c.p. 31000, Chihuahua, Chihuahua, Mexico (e-mail:luevano@infosel.net.mx).
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Author:Luevano-Flores, Eduardo; Aguirre-Madrid, Arturo
Publication:Archives of Pathology & Laboratory Medicine
Article Type:Brief Article
Geographic Code:1USA
Date:Aug 1, 2000
Words:1432
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