Indian Journal of Dermatology
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E–CASE REPORT
Year : 2013  |  Volume : 58  |  Issue : 2  |  Page : 160
Cutaneous changes in fibrous hamartoma of infancy


1 Department of Pediatric Surgery, Complejo Hospitalario Universitario de Vigo, Spain
2 Department of Pathology, Complejo Hospitalario Universitario de Vigo, Spain

Date of Web Publication5-Mar-2013

Correspondence Address:
Pilar F-Eire
Pediatric Surgery Department. Complejo Hospitalario Universitario de Vigo, Pizarro 22, 36203 Vigo
Spain
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.108089

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   Abstract 

Only a few published case of fibrous hamartoma of infancy (FHI) have commented on the changes in the overlying skin. There are descriptions of individual cases with cutaneous hyperpigmentation, hypertrichosis and hyperhidrosis. It is interesting to describe our case because, to our knowledge, the altered pigmentation of the overlying skin, with hairs on the surface, and eccrine gland hyperplasia combined with abortive hair follicles has not been reported together in association with the FHI. We recommend that all the biopsies must be excisional including overlying skin, thus the epidermal and dermal adnexal changes can be assessed in the microscopic evaluation which could have relevant implications from clinical and embryological point of view.


Keywords: Children, cutaneous changes, fibrous hamartoma


How to cite this article:
F-Eire P, Fojon JG. Cutaneous changes in fibrous hamartoma of infancy. Indian J Dermatol 2013;58:160

How to cite this URL:
F-Eire P, Fojon JG. Cutaneous changes in fibrous hamartoma of infancy. Indian J Dermatol [serial online] 2013 [cited 2019 Sep 22];58:160. Available from: http://www.e-ijd.org/text.asp?2013/58/2/160/108089

What was known? Fibrous Hamartoma of infancy is an infrequent mesenchymal lesion integrated by three tissue elements: immature mesenchyma, fibrous tissue and adipose mature tissue.



   Introduction Top


Fibrous hamartoma of infancy (FHI) is an infrequent mesenchymal lesion of a likely myofibroblastic origin with a prototypical histopathologic picture, integrated by three tissue elements-immature mesenchyma, fibrous tissue, and adipose mature tissue-intermingled in varying proportions. [1]

Until now, a few published cases have commented on the histologic changes in the overlaying skin. [2],[3] These changes correspond to altered pigmentation observed grossly, [2] clinical association with hypertrichosis [4] and eccrine gland hyperplasia. [3],[5]


   Clinical Case Top


A 4-month-old healthy white boy infant was brought to the dermatology clinic. His mother noticed a rapidly enlarging on the child's upper back on month before admission. Physical examination revealed the presence of 2 × 4 cm well-circumscribed, firm mass with a hairy and altered pigmentation on the upper back. The solitary lesion was firm, adherent to underlying tissue and had grown in the last 2 weeks. No family history of cutaneous abnormalities was elicited from the mother.

Excisional biopsy of the mass was performed. Grossly it was poorly circumscribed but hairy with firm gray-white tissue fixed to the underlying subcutaneous plane.

Histologically, the lesion was centred in the deep dermis although reached the papillary dermis and the cutaneous appendages. It was composed of three different mesenchymal tissues: Well-defined intersecting trabecule of fibrous tissue of varying size and shape, loosely textured areas consisting chiefly of immature small, round or stellate cells in a myxoid matrix, and varying amounts of mature fat.

Also the included overlying skin contained foci of eccrine hyperplasia with pronounced duct ectasia, dilated lumens with complex papillary formations and squamous metaplasia. This abnormal eccrine glands was surrounded mainly by the primitive mesenchymal tissue component of FHI [Figure 1]. Also it showed the formation of anomalous hair structures in the overlying epidermis, with microfollicles and abortive hair follicles [Figure 2].
Figure 1: The lesion is composed of three different mesenchymal tissues: Well - defined intersecting trabeculae of fibrous tissue, areas of immature cells in a myxoid matrix, and varying amounts of mature fat. Also the included overlying skin contained foci of eccrine hyperplasia with pronounced duct ectasia, dilated lumens with complex papillary formations and squamous metaplasia. This abnormal eccrine glands was surrounded mainly by the primitive mesenchymal tissue (H and E stain, ×400)

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Figure 2: Here it is showed the formation of anomalous hair structures in the overlying epidermis, with microfollicles and abortive hair follicles (H and E stain, ×400)

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   Discussion Top


The actual denomination of FHI was proposed by Enzinger in 1965. [1] The FHI is a soft tissue tumour from a monoclonal origin and therefore it likely represents a true neoplasia. [6],[7] In a review of 197 cases on FHI by Dickey and Sotelo-Avila, 91% arised within the first 12 months of life and 23% of them presented at birth like congenital lesions. [8]

Most cases present as solitary masses but multiple separate synchronous lesions have been reported rarely [9] Predominant areas include the axilae, shoulders, upper arms and upper trunk, although other sites have been reported. [8]

The complete excision is the treatment of choice, but incomplete excision it has a low recurrence rate between 10-15%. [2],[10] There is no evidence of malignant transformation or spontaneous regression, but long-term follow up indicates a benign biological behavior. [2] The differential diagnoses to be considered are lipofibromatosis, myofibroma, fibromatosis, infantile fibrosarcoma and so on. [1]

Only a few published case of FHI have commented on the changes in the overlying skin. There are descriptions of individual cases with cutaneous hyperpigmentation, [2] hypertrichosis [4] and hyperhidrosis. [5] The most exhaustive report in this topic is a series of 12 patients with FHI settled in the cutaneous changes in this disease. [3] In all the 5 cases that included the overlying skin presented eccrine changes described what hyperplasia, duct dilatation, intraluminal papillary formations, and squamous syringometaplasia. [3]

Some authors have postulated a direct relation between the degree of inductive epidermal changes and the distance between the dermal mesenchymal proliferation and the epidermis, [10] whereas other investigators failed to confirm these findings. [11] Although the pathogenesis of the induction changes in the epidermis overlying dermatofibromas and other mesenchymal proliferations are not known, mediators or organizers released from mesenchymall cells of the dermis may be responsible for the epidermal changes, [10],[12],[13]

It is interesting to describe our case because, to our knowledge, the altered pigmentation of the overlying skin, with hairs on the surface, and eccrine gland hyperplasia combined with abortive hair follicles has not been reported together in association with the FHI.

Following our case, we recommend that all the biopsies must be excisional including overlying skin, thus the epidermal and dermal adnexal changes can be assessed in the microscopic evaluation which could have relevant implications from clinical and embryological point of view. [3]

 
   References Top

1.Weiss SW, Goldblum WJ. Fibrous tumors of infancy and childhood. In: Weiss S, Goldblum W, JR, editors. Enzinger and Weiss's soft tissue tumors, 5 th ed. St Louis.: Mosby; 2008. p. 257-61.  Back to cited text no. 1
    
2.Sotelo-Avila C, Bale PM. Subdermal fibrous hamartoma of infancy: Pathology of 40 cases and differential diagnosis. Pediatr Pathol 1994;14:39-52.  Back to cited text no. 2
[PUBMED]    
3.Grynspan D, Meir K, Senger C, Ball NJ. Cutaneous changes in fibrous hamartoma of infancy. J Cutan Pathol 2007;34:39-43.  Back to cited text no. 3
[PUBMED]    
4.Scott DM, Peña JR, Omura EF. Fibrous hamartoma of infancy. J Am Acad Darmatol 1999;41:857-9.  Back to cited text no. 4
    
5.Weinberger MS, Pransky SM, Krous HF. Fibrous hamartoma of infancy presenting as a perspiring neck mass. Int J Pediatr Otorhinolaryngol 1993;26:173-6.  Back to cited text no. 5
[PUBMED]    
6.Rougemont AL, Fetni R, Murthy S, Fournet JC. A complex translocation (6;12;8) (q25;q24.3;q13) in a fibrous hamartoma of infancy. Cancer Genet Cytogenet 2006;171:115-8.  Back to cited text no. 6
[PUBMED]    
7.Lakshminarayanan R, Konia T, Welborn J. Fibrous hamartoma of infancy: A case report with associated cytogenetic findings. Arch Pathol Lab Med 2005;129:520-2.  Back to cited text no. 7
[PUBMED]    
8.Dickey GE, Sotelo-Avila C. Fibrous hamartoma of infancy: Current review. Pediatr Dev Pathol 1999;2:236-43.  Back to cited text no. 8
[PUBMED]    
9.Imaji R, Goto T, Takahashi Y, Akiyama T, Yamadori I. A case of recurrent and synchronous fibrous hamartoma of infancy. Pediatr Surg Int 2005;21:119-20.  Back to cited text no. 9
[PUBMED]    
10.Carretto E, Dall'Igna P, Alaggio R, Siracusa F, Granata C, Ferrari A, et al. Fibrous hamartoma of infancy: An Italian multi-institutional experience. J Am Acad Dermatol 2006;54:800-3.  Back to cited text no. 10
[PUBMED]    
11.Dalziel K, Marks R. Hair follicle-like change over histiocytomas. Am J Dermatopathol 1986;8:462-6.  Back to cited text no. 11
[PUBMED]    
12.Requena L, Yus ES, Simón P, del Rio E. Induction of cutaneous hyperplasias by altered stroma. Am J Dermatopathol 1996;18:248-68.  Back to cited text no. 12
    
13.Francis AJ, Marks R. The effects of anti-prostaglandin agents on epidermal proliferation induced by dermal inflammation. Br J Dermatol 1977;97:395-400.  Back to cited text no. 13
[PUBMED]    

What is new? To our knowledge the altered pigmentation of the overlying skin, with hairs on the surface and eccrine gland hyperplasia combined with abortive hair follicles has not been reported together in association with Fibrous Hamartoma of infancy.


    Figures

  [Figure 1], [Figure 2]



 

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