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Soft tissue Tumors

Fibroblastic / myofibroblastic

Fibromatosis colli


Reviewer: Komal Arora, M.D. (see Reviewers page)
Revised: 20 July 2012, last major update July 2012
Copyright: (c) 2003-2012, PathologyOutlines.com, Inc.

General
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● Fibromatosis that appears at birth, often bilateral, affecting lower 1/3 of sternocleidomastoid muscle, causing thickened muscle
● Also called congenital torticollis (torticollis: twisting of neck causing unnatural position of head, usually caused by spasm of neck muscles, Am Fam Physician 1996;54:1965)

Epidemiology
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● Associated with congenital anomalies (14% have congenital dislocations of hip, also breech deliveries)
● May be due to birth injury (breech presentation, forceps)
● Uncommon (0.4% of live births), usually diagnosed by age 6 months
● Recommended to diagnose by FNA since excision usually is not required

Treatment
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Early - stretching and physiotherapy, resolves in 70%
● Some cases require resection of affected muscle
● Does not recur

Gross description
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● Tan gritty mass of muscle up to 3 cm, no hemorrhage or necrosis

Micro description
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● Diffuse proliferation of uniform plump fibroblasts and myofibroblasts and scar like collagen in muscle, with entrapped reactive and degenerating skeletal muscle fibers (loss of cross striations, nuclear enlargement and hypercellularity, multinucleation, atrophy)
● Surgical specimens are usually less cellular than FNA specimens because they are obtained later in time course of disease

Micro images
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Multinodular proliferation of acellular collagenized tissue replaces part of sternocleidomastoid muscle


Skeletal muscle fibers are trapped at advancing edge of lesion


Scattered, bland fibrocytes are widely separated by dense collagen

Cytology
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Early - cellular specimen with clusters or parallel arrays of bland appearing spindle cells in fibromyxoid matrix
● Also atrophic skeletal muscle in clean background, frequent muscle giant cells, bland bare nuclei and collagen (Acta Cytol 2003;47:359)
● Usually no significant inflammation (Diagn Cytopathol 2000;23:338)

Positive stains
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● Vimentin, actins

Differential diagnosis
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● Fibromatosis: no muscle fibers which are replaced by fibrous tissue except at periphery, does not typically affect sternocleidomastoid muscle
● Proliferative myositis: doesn’t affect this site, stroma resembles granulation tissue and is not collagenous
● Fibrodysplasia ossificans progressiva: doesn’t affect this site, hand malformations are present, bone is present

End of Soft Tissue Tumors > Fibroblastic / myofibroblastic > Fibromatosis colli


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