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Benign Lesion on the Posterior Aspect of the Neck
Nuchal-Type Fibroma
Nuchal-type fibroma (NTF) is a rare benign proliferation of the dermis and subcutis associated with diabetes mellitus and Gardner syndrome.1,2 Forty-four percent of patients with NTF have diabetes mellitus.2 The posterior aspect of the neck is the most frequently affected site, but lesions also may present on the upper back, lumbosacral area, buttocks, and face. Physical examination generally reveals an indurated, asymptomatic, ill-defined, 3-cm or smaller nodule that is hard and white, unencapsulated, and poorly circumscribed.
Histopathologic examination of NTF typically reveals a nodular paucicellular proliferation of thick collagen bundles with inconspicuous fibroblasts, radiation of collagenous septa into the subcutaneous fat, and entrapment of mature adipose tissue and small nerves (quiz image A). Collagen bundles are thickened with entrapment of adipose tissue without increased cellularity (quiz image B). S-100 staining can show the entrapped nerves.
Similar to NTF, sclerotic fibroma is a firm dermal nodule with histologic examination usually demonstrating a paucicellular collagenous tumor. In sclerotic fibromas, the collagen pattern resembles Vincent van Gogh’s painting “The Starry Night” and may be a marker for Cowden disease (Figure 1).3 Solitary fibrous tumors are distinguished by more hypercellular areas, patternless pattern, and staghorn-shaped blood vessels (Figure 2).4 Spindle cell lipoma classically demonstrates a mixture of mature adipocytes and bland spindle cells in a mucinous or fibrous background with thick collagen bundles with no storiform pattern (Figure 3). Some variants of spindle cell lipoma have minimal or no fat.5 All of these conditions have positive immunohistochemical staining for CD34.
However, dermatofibroma is CD34‒. Dermatofibroma is characterized by an interstitial spindle cell proliferation with a loose storiform pattern, collagen trapping at the outer edges of the tumor, overlying platelike acanthosis, and sometimes follicular induction (Figure 4).
Nuchal-type fibroma also can resemble scleredema. Both lesions can show increased and thickened collagen bundles without notable fibroblast proliferation; the difference is the occurrence of mucin in scleredema. However, incases of late-stage scleredema, mucin is not always demonstrated. Therefore, one can conclude that histologically NTF is closely associated with late-stage scleredema.6
- Dawes LC, La Hei ER, Tobias V, et al. Nuchal fibroma should be recognized as a new extracolonic manifestation of Gardner-variant familial adenomatous polyposis. Aust N Z J Surg. 2000;70:824-826.
- Michal M, Fetsch JF, Hes O, et al. Nuchal-type fibroma: a clinicopathologic study of 52 cases. Cancer. 1999;85:156-163.
- Pernet C, Durand L, Bessis D, et al. Solitary sclerotic fibroma of the skin: a possible clue for Cowden syndrome. Eur J Dermatol. 2012;22:278-279.
- Omori Y, Saeki H, Ito K, et al. Solitary fibrous tumour of the scalp. Clin Exp Dermatol. 2014;39:539-541.
- Billings SD, Folpe AL. Diagnostically challenging spindle cell lipomas: a report of 34 “low-fat” and “fat-free” variants. Am J Dermatopathol. 2007;29:437-442.
- Banney LA, Weedon D, Muir JB. Nuchal fibroma associated with scleredema, diabetes mellitus and organic solvent exposure. Australas J Dermatol. 2000;41:39-41.
Nuchal-Type Fibroma
Nuchal-type fibroma (NTF) is a rare benign proliferation of the dermis and subcutis associated with diabetes mellitus and Gardner syndrome.1,2 Forty-four percent of patients with NTF have diabetes mellitus.2 The posterior aspect of the neck is the most frequently affected site, but lesions also may present on the upper back, lumbosacral area, buttocks, and face. Physical examination generally reveals an indurated, asymptomatic, ill-defined, 3-cm or smaller nodule that is hard and white, unencapsulated, and poorly circumscribed.
Histopathologic examination of NTF typically reveals a nodular paucicellular proliferation of thick collagen bundles with inconspicuous fibroblasts, radiation of collagenous septa into the subcutaneous fat, and entrapment of mature adipose tissue and small nerves (quiz image A). Collagen bundles are thickened with entrapment of adipose tissue without increased cellularity (quiz image B). S-100 staining can show the entrapped nerves.
Similar to NTF, sclerotic fibroma is a firm dermal nodule with histologic examination usually demonstrating a paucicellular collagenous tumor. In sclerotic fibromas, the collagen pattern resembles Vincent van Gogh’s painting “The Starry Night” and may be a marker for Cowden disease (Figure 1).3 Solitary fibrous tumors are distinguished by more hypercellular areas, patternless pattern, and staghorn-shaped blood vessels (Figure 2).4 Spindle cell lipoma classically demonstrates a mixture of mature adipocytes and bland spindle cells in a mucinous or fibrous background with thick collagen bundles with no storiform pattern (Figure 3). Some variants of spindle cell lipoma have minimal or no fat.5 All of these conditions have positive immunohistochemical staining for CD34.
However, dermatofibroma is CD34‒. Dermatofibroma is characterized by an interstitial spindle cell proliferation with a loose storiform pattern, collagen trapping at the outer edges of the tumor, overlying platelike acanthosis, and sometimes follicular induction (Figure 4).
Nuchal-type fibroma also can resemble scleredema. Both lesions can show increased and thickened collagen bundles without notable fibroblast proliferation; the difference is the occurrence of mucin in scleredema. However, incases of late-stage scleredema, mucin is not always demonstrated. Therefore, one can conclude that histologically NTF is closely associated with late-stage scleredema.6
Nuchal-Type Fibroma
Nuchal-type fibroma (NTF) is a rare benign proliferation of the dermis and subcutis associated with diabetes mellitus and Gardner syndrome.1,2 Forty-four percent of patients with NTF have diabetes mellitus.2 The posterior aspect of the neck is the most frequently affected site, but lesions also may present on the upper back, lumbosacral area, buttocks, and face. Physical examination generally reveals an indurated, asymptomatic, ill-defined, 3-cm or smaller nodule that is hard and white, unencapsulated, and poorly circumscribed.
Histopathologic examination of NTF typically reveals a nodular paucicellular proliferation of thick collagen bundles with inconspicuous fibroblasts, radiation of collagenous septa into the subcutaneous fat, and entrapment of mature adipose tissue and small nerves (quiz image A). Collagen bundles are thickened with entrapment of adipose tissue without increased cellularity (quiz image B). S-100 staining can show the entrapped nerves.
Similar to NTF, sclerotic fibroma is a firm dermal nodule with histologic examination usually demonstrating a paucicellular collagenous tumor. In sclerotic fibromas, the collagen pattern resembles Vincent van Gogh’s painting “The Starry Night” and may be a marker for Cowden disease (Figure 1).3 Solitary fibrous tumors are distinguished by more hypercellular areas, patternless pattern, and staghorn-shaped blood vessels (Figure 2).4 Spindle cell lipoma classically demonstrates a mixture of mature adipocytes and bland spindle cells in a mucinous or fibrous background with thick collagen bundles with no storiform pattern (Figure 3). Some variants of spindle cell lipoma have minimal or no fat.5 All of these conditions have positive immunohistochemical staining for CD34.
However, dermatofibroma is CD34‒. Dermatofibroma is characterized by an interstitial spindle cell proliferation with a loose storiform pattern, collagen trapping at the outer edges of the tumor, overlying platelike acanthosis, and sometimes follicular induction (Figure 4).
Nuchal-type fibroma also can resemble scleredema. Both lesions can show increased and thickened collagen bundles without notable fibroblast proliferation; the difference is the occurrence of mucin in scleredema. However, incases of late-stage scleredema, mucin is not always demonstrated. Therefore, one can conclude that histologically NTF is closely associated with late-stage scleredema.6
- Dawes LC, La Hei ER, Tobias V, et al. Nuchal fibroma should be recognized as a new extracolonic manifestation of Gardner-variant familial adenomatous polyposis. Aust N Z J Surg. 2000;70:824-826.
- Michal M, Fetsch JF, Hes O, et al. Nuchal-type fibroma: a clinicopathologic study of 52 cases. Cancer. 1999;85:156-163.
- Pernet C, Durand L, Bessis D, et al. Solitary sclerotic fibroma of the skin: a possible clue for Cowden syndrome. Eur J Dermatol. 2012;22:278-279.
- Omori Y, Saeki H, Ito K, et al. Solitary fibrous tumour of the scalp. Clin Exp Dermatol. 2014;39:539-541.
- Billings SD, Folpe AL. Diagnostically challenging spindle cell lipomas: a report of 34 “low-fat” and “fat-free” variants. Am J Dermatopathol. 2007;29:437-442.
- Banney LA, Weedon D, Muir JB. Nuchal fibroma associated with scleredema, diabetes mellitus and organic solvent exposure. Australas J Dermatol. 2000;41:39-41.
- Dawes LC, La Hei ER, Tobias V, et al. Nuchal fibroma should be recognized as a new extracolonic manifestation of Gardner-variant familial adenomatous polyposis. Aust N Z J Surg. 2000;70:824-826.
- Michal M, Fetsch JF, Hes O, et al. Nuchal-type fibroma: a clinicopathologic study of 52 cases. Cancer. 1999;85:156-163.
- Pernet C, Durand L, Bessis D, et al. Solitary sclerotic fibroma of the skin: a possible clue for Cowden syndrome. Eur J Dermatol. 2012;22:278-279.
- Omori Y, Saeki H, Ito K, et al. Solitary fibrous tumour of the scalp. Clin Exp Dermatol. 2014;39:539-541.
- Billings SD, Folpe AL. Diagnostically challenging spindle cell lipomas: a report of 34 “low-fat” and “fat-free” variants. Am J Dermatopathol. 2007;29:437-442.
- Banney LA, Weedon D, Muir JB. Nuchal fibroma associated with scleredema, diabetes mellitus and organic solvent exposure. Australas J Dermatol. 2000;41:39-41.
The best diagnosis is:
a. dermatofibroma
b. nuchal-type fibroma
c. sclerotic fibroma
d. solitary fibrous tumor
e. spindle cell lipoma
Continue to the next page for the diagnosis >>
