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 Table of Contents  
CASE REPORTS
Year : 2021  |  Volume : 8  |  Issue : 2  |  Page : 61-63

Nuchal fibroma in a diabetic patient: Rare case report with histopathological features


Department of Pathology, Bhabha Atomic Research Centre Hospital, Anushakti Nagar Mumbai 400094, India

Date of Submission07-Jan-2021
Date of Decision30-Jun-2021
Date of Acceptance23-Jul-2021
Date of Web Publication06-Oct-2022

Correspondence Address:
Prachi Ramkrishna Gaddam
Department of Pathology, BARC Hospital, Anushakti Nagar, Mumbai 400094
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijdpdd.ijdpdd_1_21

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  Abstract 

Nuchal fibroma is a benign tumor-like proliferation which presents as asymptomatic, slow-growing solitary swelling located in the lower back and the neck with microscopic findings of collagen fibers and entrapped adnexae. It occurs more frequently in males between 25 and 60 years. It is associated with diabetes mellitus, Gardner’s syndrome, and trauma. Histopathological examination and differentiation of nuchal fibroma from other soft tissue tumors over the posterior neck are essential for the accurate diagnosis. Complete excision and follow-up are necessary for proper management. We report a rare case of nuchal fibroma presenting as posterior neck swelling in a 58-year-old diabetic patient.

Keywords: Collagenous, fibroma, nuchal


How to cite this article:
Gaddam PR, Sruthi M, Cherian S. Nuchal fibroma in a diabetic patient: Rare case report with histopathological features. Indian J Dermatopathol Diagn Dermatol 2021;8:61-3

How to cite this URL:
Gaddam PR, Sruthi M, Cherian S. Nuchal fibroma in a diabetic patient: Rare case report with histopathological features. Indian J Dermatopathol Diagn Dermatol [serial online] 2021 [cited 2023 Feb 8];8:61-3. Available from: https://www.ijdpdd.com/text.asp?2021/8/2/61/357929




  Introduction Top


Nuchal fibroma also known as nuchal-type fibroma, collagenosis nuchae, is a benign tumor-like proliferation which presents as asymptomatic, slow-growing solitary swelling.[1] This lesion arises from the posterior cervical subcutaneous tissue and occurs more frequently in males between 25 and 60 years.[2] It is associated with diabetes mellitus, Gardner’s syndrome, and trauma.[3] Nuchal-type fibroma was described first by Enzinger and Weiss[5] in 1988. Thus far, only rare case reports and clinicopathological case series of around 100 cases have been reported in the literature.[1] Histologically, the lesion consists of hypocellular, dense collagen bundles with very few interspersed fibroblasts. This lesion can be easily misdiagnosed due to overlapping histopathological features.[4] We report a rare case of nuchal fibroma with detailed histopathological features in a 58-year-old diabetic patient presenting with posterior neck swelling.


  Case History Top


A 58-year-old male with history of diabetes mellitus since 7 years presented with swelling at the nape of the neck with pain radiating to the vertex. The swelling was slowly growing to the present size of 3 cm diameter in a span of 7 months. There was no history of trauma/skin lesion. Routine blood investigations were within normal limits. Ultrasonography revealed predominantly hypoechoic mass lesion in subcutaneous plane in the nape of neck. Fine needle aspiration cytology was performed which revealed only blood and occasional collagenous tissue fragments. Subsequently, the patient underwent wide local excision of the tumor. The gross examination of the specimen showed a poorly circumscribed, yellowish tan, firm mass with fatty and fibrous areas measuring 3.8 × 3 × 2.8 cm. Microscopic examination revealed a non-encapsulated, ill-defined, hypocellular lesion with haphazardly arranged collagen bundles with scant mature fibroblasts [Figure 1]. Entrapped adipose tissue, blood vessels, adnexal structures, and nerve bundles with surrounding fibrosis were seen amidst the collagen bundles [Figure 2]A-C]. There were no necrosis and mitoses.
Figure 1: Histology showing randomly oriented dense collagen bundles with interspersed adipose tissue (H&E,100×)

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Figure 2: A. Entrapped adipose tissue, nerves, and blood vessels amidst dense collagen bundles. B. Proliferative entrapped nerve fibers (arrows). C. Encased adnexal structures (H&E, A: 50×, B: 200×, C: 100×)

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  Discussion Top


Nuchal-type fibroma is a rare proliferation involving dermis and subcutaneous tissue, which typically presents as a solitary unencapsulated tumor in the cervicodorsal region.[5 Similar lesions are reported at extra nuchal sites as well],[ hence the term “nuchal-type fibroma” was proposed.[6] Reports have shown a link to Gardner’s syndrome, and such lesions are referred to as Gardner-associated fibromas.[5] Michal et al.[2] reported association of nuchal fibroma with Gardner’s syndrome in their case series. This lesion is associated with diabetes mellitus in 44% of the cases.[5] Suggested pathogenesis of diabetes-associated nuchal fibroma is due to excessive deposition of collagen due to increased collagen synthesis mediated by elevated levels of transforming growth factor-b, decreased collagen resorption, or a combination of both.[2] Normal tissue from the nuchal area shows the presence of dense paucicellular collagenous connective tissue. Nuchal-type fibroma represents a localized accentuation leading to expansion of the collagenized dermis with focal lobular architecture, effacement of the subcutis.[2] Paucicellular nature and tendency of recurrence in nuchal-type fibroma suggest a peculiar tumor-like reactive process due to persistence of local or systemic factors that initially triggered its development.[2] Nuchal-type fibroma commonly occurs in men, with a peak incidence in the third to fifth decades.[5] Clinically, the lesion is usually asymptomatic and slow growing with a non-tender, mobile, poorly circumscribed tumor in the subcutaneous tissues of the posterior neck or occipital region. When it is symptomatic, pain is the most common complaint with restricted range of movements of the cervical spine and upper limb.[7] Fine needle aspiration cytology may not provide satisfactory diagnosis as hypocellular nature of this lesion will lead to lack of diagnostic material.[7] Histopathological examination is essential for the diagnosis. Microscopy reveals sheets of broad hypocellular fibro collagenous bundles in subcutis with few interspersed mature fibroblasts. The sparsely scattered fibroblasts have scant cytoplasm and elongated, twisted, and tapered nuclei with a single inconspicuous nucleolus. Encasement of adnexa, with entrapped adipocytes, nerves, and vessels with minimal perivascular inflammation are peculiar histological features. The peripheral nerve twigs exhibit splayed appearance or perineural fibrosis.[4] The tumor commonly radiates into the surrounding fatty tissue in multiple short processes, and extension into the underlying skeletal muscle is observed in some cases.[2] Elastic stains show scarce, morphologically normal appearing elastic fibers.[7] Gong et al.[8] have reported mucoid tissue degeneration in a case of nuchal-type fibroma. The spindle cells are positive for Vimentin and CD34 and negative for smooth muscle actin and Desmin. Sixty-four percent of the cases show a nuclear reaction with B-catenin. Some cases show positive staining with CD99.[4],[5]

Histopathological differentiation of nuchal fibroma from other soft tissue tumors arising in the posterior neck is essential for accurate diagnosis.[4] Fibrolipoma is the most common differential diagnosis. Encapsulated lesion with greater proportion of mature adipose tissue and lack of entrapped nerves differentiates it from fibrolipoma. Subcutaneous location and paucity of cells and lack of cartilaginous metaplasia can differentiate nuchal fibroma from extra-abdominal fibromatosis and nuchal fibrocartilaginous pseudotumor.[5] Elastofibroma arises in the deep soft tissue of the scapula in the inferomedial portion and contains characteristic fragmented elastic fibers. Keloid scar develops as reaction to injury and has denser, thicker collagen than nuchal-type fibroma.[9]

Many cases of nuchal-type fibroma have been misdiagnosed or not recognized because of its indolent clinical course and histopathological similarity to other benign fibrous tumors.[10] Careful total excision is necessary for the treatment and accurate diagnosis. Nuchal-type fibroma is benign but may recur if incompletely excised and hence follow-up is required.[5]


  Conclusion Top


The true incidence of nuchal-type fibroma is probably higher than recognized, and it should be considered as a potential diagnosis for a subcutaneous swelling in the posterior neck with microscopic findings of typical collagen fibers and entrapped adnexae. The present report highlights histopathological features and differential diagnoses of this uncommon lesion, as complete excision and follow-up are necessary for clinical management.

Authors’ contribution

Gaddam PR and Sruthi M: Concept and design of study or acquisition of data or analysis and interpretation of data; Gaddam PR, Sruthi M, and Cherian S: drafting the article or revising it critically for important intellectual content; Gaddam PR and Cherian S: final approval of the version to be published.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Kostakis ID, Feretis T, Damaskos C, Garmpis N, Liapis G, Pateras I, et al. Nuchal-type fibroma: Single-center experience and systematic literature review. In Vivo 2020;34:2217-23.  Back to cited text no. 1
    
2.
Michal M, Fetsch JF, Hes O, Miettinen M Nuchal-type fibroma: A clinicopathologic study of 52 cases. Cancer 1999;85:156-63.  Back to cited text no. 2
    
3.
Lee CC, Lai CS, Lin CH, Lin YN, Chu SC, Chai CY, et al. Extra nuchal-type fibroma associated with repetitive blunt trauma during religious activities. Trauma Case Rep 2016;4:16-20.  Back to cited text no. 3
    
4.
Alsaleh N, Amanguno H Nuchal fibroma: A rare entity of neck masses. Gulf J Oncolog 2015;1:10-2.  Back to cited text no. 4
    
5.
Enzinger FM, Weiss, SW Benign tumors and tumor-like lesions of fibrous tissue. In: Enzinger FM, Weiss SW, editors. Soft tissue tumors. 2nd ed. St Louis, MO: CV Mosby, 1988. pp. 102-35.  Back to cited text no. 5
    
6.
Shin JB, Son SW, Kim IH Nuchal-type fibroma of the coccyx. Ann Dermatol 2008;20:41-4.  Back to cited text no. 6
    
7.
Karonidis A, Rigby HS, Orlando A Collagenosis nuchae: A case report of a rare and often misdiagnosed condition. J Plast Reconstr Aesthet Surg 2007;60:320-3.  Back to cited text no. 7
    
8.
Gong Y, Zhao X, Wu DI, Liu J Nuchal-type fibroma of the shoulder: A case report and review of the literature. Oncol Lett 2016;11:4152-4.  Back to cited text no. 8
    
9.
Diwan AH, Graves ED, King JA, Horenstein MG Nuchal-type fibroma in two related patients with Gardner’s syndrome. Am J Surg Pathol 2000;24:1563-7.  Back to cited text no. 9
    
10.
Kim DH, Kim TH, Sung NH, Shin H, Lee AY, Lee SH Multiple nuchal-type fibromas on the scalp: A case report. Ann Dermatol 2015;27:194-6.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2]



 

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