Indian Journal of Pathology and Microbiology
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Year : 2010  |  Volume : 53  |  Issue : 4  |  Page : 827-828
Morphological spectrum of inclusion body fibromatosis: A rare case report


Department of Pathology, Tata Memorial Hospital, E. Borges Marg, Parel, Mumbai, India

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Date of Web Publication27-Oct-2010
 

How to cite this article:
Suryawanshi P, Rekhi B, Jambhekar NA. Morphological spectrum of inclusion body fibromatosis: A rare case report. Indian J Pathol Microbiol 2010;53:827-8

How to cite this URL:
Suryawanshi P, Rekhi B, Jambhekar NA. Morphological spectrum of inclusion body fibromatosis: A rare case report. Indian J Pathol Microbiol [serial online] 2010 [cited 2014 Oct 25];53:827-8. Available from: http://www.ijpmonline.org/text.asp?2010/53/4/827/72062


Infantile digital fibromatosis (IDF) or inclusion body fibromatosis (IBF) is a rare tumor with distinct clinicopathological features. It is invariably an intradermal, unencapsulated tumor composed of spindly cells arranged in whorls and fascicles and displays characteristic intracytoplasmic eosinophilic inclusion bodies that distinguish it from other fibromatosis. [1] Its typical presentation is in the form of an asymptomatic 'fleshy' nodule occurring on the lateral or dorsal aspect of the digits at birth or in the first few years of life. [2] The lesions rarely exceed 2 cm in size and have a tendency for spontaneous regression. Therefore, conservative treatment is generally recommended. [3] Herein, we describe morphological spectrum, including immunohistochemical (IHC) analysis of an uncommon case of an IDF in a baby girl.

A 6-month-old baby girl presented with nodules on the terminal phalanges of her toes since birth. Physical examination revealed 1 and 1.5-cm sized nodules on the 3 rd and 5 th toes of her right foot, respectively, for which she underwent excision, elsewhere. The paraffin blocks were submitted to us for review. Hematoxylin and Eosin (H and E) stained section revealed an unencapsulated polypoidal lesion consisting of attenuated epidermis with proliferation of sheets and fascicles of spindly, myofibroblastic cells beneath. On higher magnification, tumor cells revealed distinct round, eosinophilic, paranuclear inclusions [Figure 1] a, b. Masson's trichome (MT) stain highlighted the inclusions [Figure 1]c. On IHC, the tumor cells and the inclusions were positive for vimentin and smooth muscle actin (SMA) [Figure 1]d.
Figure 1: (a) Subepidermal proliferation of spindly myofibroblastic cells in sheets and fascicles. [H and E, ×200]; (b) Paranuclear eosinophilic inclusions within the spindly cells. [H and E; ×400] Inset highlights the discrete paranuclear inclusion [H and E, ×1000]; (c) Special stain highlights several red inclusions [MT, ×1000]; (d) Diffuse SMA positivity in tumor cells. [DAB, ×200] Inset highlighting SMA positive inclusions [DAB, ×1000].

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Infantile digital fibromatosis (IDF) or inclusion body fibromatosis (IBF) is a relatively rare disease with an estimated prevalence of 2.5%. [4] It was termed as multiple hyaline fibromatosis by Reye; [1] therefore designated as Reye's tumor, apart from infantile dermal fibromatosis, subdermal fibromatous tumor of infancy, recurring digital fibroma and juvenile dermatofibroma are the other designations. [1],[5] It has a distinct clinicopathological profile. It is characterized by 1 to 2 cm sized, single or multiple, firm and painless nodules restricted to the fingers and toes, [5] the later site noted in our case. Microscopic appearance of IDF or IBF includes dermal proliferation of fibroblasts and myofibroblasts arranged in interlacing fascicles and bundles. The differential diagnoses include keloids, hypertrophic scar tissue, terminal osseous dysplasia and pigmentary defects, and juvenile aponeurotic fibroma, some of which were considered in the present case. [3] Further, on careful examination with high index of suspicion one can appreciate juxtanuclear 3-10 μm sized round paranuclear inclusion bodies in the myofibroblastic cells that are highlighted with special stains, as seen in our case. [3],[5],[6] Current IHC and ultrastructural studies suggest that the inclusion bodies are composed of actin and vimentin, [3] a feature substantiated and uniquely elucidated in our case with strong SMA positivity.

Clinically spontaneous regression in an IDF is known; therefore, conservative treatment is generally recommended. [3],[5] Surgery is reserved for cases of aggressive growth or functional impairment. Although, lesions recur greater than 60% of the time following excision; [3] the ultimate prognosis is excellent. This tumor does not metastasize. In our case, the lesion recurred after 4 months of excision and the patient has been recommended further follow-up.

 
   References Top

1.Reye RD. Recurring digital fibrous tumors of childhood. Arch Pathol 1965;80:228-31.  Back to cited text no. 1
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2.Chirayil PT, Jayaraj J, Kumar P. Infantile digital fibromatosis: A case report. Burns 2001;27:89-90.  Back to cited text no. 2
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3.Niamba P, Léauté-Labrèze C, Boralevi F, Lepreux S, Chamaillard M, Vergnes P, et al. Further documentation of spontaneous regression of infantile digital fibromatosis. Pediatr Dermatol 2007;24:280-4.  Back to cited text no. 3
    
4.Coffin CM, Dehner LP. Fibroblastic-myofibroblastic tumors in children and adolescents: A clinicopathologic study of 108 examples in 103 patients. Pediatr Pathol 1991;11:569-88.  Back to cited text no. 4
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5.Grenier N, Liang C, Capaldi L, Ney A, Lapidus C, Schappell D, et al. A range of histologic findings in infantile digital fibromatosis. Pediatr Dermatol 2008;25:72-5.   Back to cited text no. 5
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6.Kanwar AJ, Kaur S, Thami GP, Mohan H. Congenital infantile digital fibromatosis. Pediatr Dermatol 2002;19:370.  Back to cited text no. 6
[PUBMED]  [FULLTEXT]  

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Correspondence Address:
Bharat Rekhi
Department of Pathology, Tata Memorial Hospital, E. Borges Marg, Parel, Mumbai - 400 012
India
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DOI: 10.4103/0377-4929.72062

PMID: 21045432

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