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Multiple Asymptomatic Dome-Shaped Papules on the Scalp
The Diagnosis: Spiradenocylindroma
Shave biopsies of our patient’s lesions showed wellcircumscribed dermal nodules resembling a spiradenoma with 3 cell populations: those with lighter nuclei, darker nuclei, and scattered lymphocytes. However, the conspicuous globules of basement membrane material were reminiscent of a cylindroma. These overlapping features and the patient’s history of cylindroma were suggestive of a diagnosis of spiradenocylindroma.
Spiradenocylindroma is an uncommon dermal tumor with features that overlap with spiradenoma and cylindroma.1 It may manifest as a solitary lesion or multiple lesions and can occur sporadically or in the context of a family history. Histologically, it must be distinguished from other intradermal basaloid neoplasms including conventional cylindroma and spiradenoma, dermal duct tumor, hidradenoma, and trichoblastoma.
When patients present with multiple cylindromas, spiradenomas, or spiradenocylindromas, physicians should consider genetic testing and review of the family history to assess for cylindromatosis gene mutations or Brooke-Spiegler syndrome. Biopsy and histologic examination are important because malignant tumors can evolve from pre-existing spiradenocylindromas, cylindromas, and spiradenomas,2 with an increased risk in patients with Brooke-Spiegler syndrome.1 Our patient declined further genetic workup but continues to follow up with dermatology for monitoring of lesions.
Dermal duct tumors are morphologic variants of poromas that are derived from sweat gland lineage and usually manifest as solitary dome-shaped papules, plaques, or nodules most often seen on acral surfaces as well as the head and neck.3 Clinically, they may be indistinguishable from spiradenocylindromas and require biopsy for histologic evaluation. They can be distinguished from spiradenocylindroma by the presence of small dermal nodules composed of cuboidal cells with ample pink cytoplasm and cuticle-lined ducts (Figure 1).
Trichoblastomas typically are deep-seated basaloid follicular neoplasms on the scalp with papillary mesenchyme resembling the normal fibrous sheath of the hair follicle, often replete with papillary mesenchymal bodies (Figure 2). There generally are no retraction spaces between its basaloid nests and the surrounding stroma, which is unlikely to contain mucin relative to basal cell carcinoma (BCC).4,5
Adenoid cystic carcinoma is a rare salivary gland tumor that can metastasize to the skin and rarely arises as a primary skin adnexal tumor. It manifests as a slowgrowing mass that can be tender to palpation.6 Histologic examination shows dermal islands with cribriform blue and pink spaces. Compared to BCC, adenoid cystic carcinoma cells are enlarged and epithelioid with relatively scarce cytoplasm (Figure 3).6,7 Adenoid cystic carcinoma can show variable growth patterns including infiltrative nests and trabeculae. Perineural invasion is common, and there is a high risk for local recurrence.7 First-line therapy usually is surgical, and postoperative radiotherapy may be required.6,7
Nodular BCC commonly manifests as an enlarging nonhealing lesion on sun-exposed skin and has many subtypes, typically with arborizing telangiectases on dermoscopy. Histopathologic examination of nodular BCC reveals a nest of basaloid follicular germinative cells in the dermis with peripheral palisading and a fibromyxoid stroma (Figure 4).8 Patients with Brooke-Spiegler syndrome are at increased risk for nodular BCC, which may be clinically indistinguishable from spiradenoma, cylindroma, and spiradenocylindroma, necessitating histologic assessment.
- Facchini V, Colangeli W, Bozza F, et al. A rare histopathological spiradenocylindroma: a case report. Clin Ter. 2022;173:292-294. doi:10.7417/ CT.2022.2433
- Kazakov DV. Brooke-Spiegler syndrome and phenotypic variants: an update [published online March 14, 2016]. Head Neck Pathol. 2016;10:125-30. doi:10.1007/s12105-016-0705-x
- Miller AC, Adjei S, Temiz LA, et al. Dermal duct tumor: a diagnostic dilemma. Dermatopathology (Basel). 2022;9:36-47. doi:10.3390/dermatopathology9010007
- Elston DM. Pilar and sebaceous neoplasms. In: Elston DM, Ferringer T, Ko C, et al. Dermatopathology. 3rd ed. Elsevier; 2018:71-85.
- McCalmont TH, Pincus LB. Adnexal neoplasms. In: Bolognia J, Schaffer J, Cerroni, L. Dermatology. 4th ed. Elsevier; 2017:1930-1953.
- Coca-Pelaz A, Rodrigo JP, Bradley PJ, et al. Adenoid cystic carcinoma of the head and neck—an update [published online May 2, 2015]. Oral Oncol. 2015;51:652-661. doi:10.1016/j.oraloncology.2015.04.005
- Tonev ID, Pirgova YS, Conev NV. Primary adenoid cystic carcinoma of the skin with multiple local recurrences. Case Rep Oncol. 2015;8:251- 255. doi:10.1159/000431082
- Cameron MC, Lee E, Hibler BP, et al. Basal cell carcinoma: epidemiology; pathophysiology; clinical and histological subtypes; and disease associations [published online May 18, 2018]. J Am Acad Dermatol. 2019;80:303-317. doi:10.1016/j.jaad.2018.03.060
The Diagnosis: Spiradenocylindroma
Shave biopsies of our patient’s lesions showed wellcircumscribed dermal nodules resembling a spiradenoma with 3 cell populations: those with lighter nuclei, darker nuclei, and scattered lymphocytes. However, the conspicuous globules of basement membrane material were reminiscent of a cylindroma. These overlapping features and the patient’s history of cylindroma were suggestive of a diagnosis of spiradenocylindroma.
Spiradenocylindroma is an uncommon dermal tumor with features that overlap with spiradenoma and cylindroma.1 It may manifest as a solitary lesion or multiple lesions and can occur sporadically or in the context of a family history. Histologically, it must be distinguished from other intradermal basaloid neoplasms including conventional cylindroma and spiradenoma, dermal duct tumor, hidradenoma, and trichoblastoma.
When patients present with multiple cylindromas, spiradenomas, or spiradenocylindromas, physicians should consider genetic testing and review of the family history to assess for cylindromatosis gene mutations or Brooke-Spiegler syndrome. Biopsy and histologic examination are important because malignant tumors can evolve from pre-existing spiradenocylindromas, cylindromas, and spiradenomas,2 with an increased risk in patients with Brooke-Spiegler syndrome.1 Our patient declined further genetic workup but continues to follow up with dermatology for monitoring of lesions.
Dermal duct tumors are morphologic variants of poromas that are derived from sweat gland lineage and usually manifest as solitary dome-shaped papules, plaques, or nodules most often seen on acral surfaces as well as the head and neck.3 Clinically, they may be indistinguishable from spiradenocylindromas and require biopsy for histologic evaluation. They can be distinguished from spiradenocylindroma by the presence of small dermal nodules composed of cuboidal cells with ample pink cytoplasm and cuticle-lined ducts (Figure 1).
Trichoblastomas typically are deep-seated basaloid follicular neoplasms on the scalp with papillary mesenchyme resembling the normal fibrous sheath of the hair follicle, often replete with papillary mesenchymal bodies (Figure 2). There generally are no retraction spaces between its basaloid nests and the surrounding stroma, which is unlikely to contain mucin relative to basal cell carcinoma (BCC).4,5
Adenoid cystic carcinoma is a rare salivary gland tumor that can metastasize to the skin and rarely arises as a primary skin adnexal tumor. It manifests as a slowgrowing mass that can be tender to palpation.6 Histologic examination shows dermal islands with cribriform blue and pink spaces. Compared to BCC, adenoid cystic carcinoma cells are enlarged and epithelioid with relatively scarce cytoplasm (Figure 3).6,7 Adenoid cystic carcinoma can show variable growth patterns including infiltrative nests and trabeculae. Perineural invasion is common, and there is a high risk for local recurrence.7 First-line therapy usually is surgical, and postoperative radiotherapy may be required.6,7
Nodular BCC commonly manifests as an enlarging nonhealing lesion on sun-exposed skin and has many subtypes, typically with arborizing telangiectases on dermoscopy. Histopathologic examination of nodular BCC reveals a nest of basaloid follicular germinative cells in the dermis with peripheral palisading and a fibromyxoid stroma (Figure 4).8 Patients with Brooke-Spiegler syndrome are at increased risk for nodular BCC, which may be clinically indistinguishable from spiradenoma, cylindroma, and spiradenocylindroma, necessitating histologic assessment.
The Diagnosis: Spiradenocylindroma
Shave biopsies of our patient’s lesions showed wellcircumscribed dermal nodules resembling a spiradenoma with 3 cell populations: those with lighter nuclei, darker nuclei, and scattered lymphocytes. However, the conspicuous globules of basement membrane material were reminiscent of a cylindroma. These overlapping features and the patient’s history of cylindroma were suggestive of a diagnosis of spiradenocylindroma.
Spiradenocylindroma is an uncommon dermal tumor with features that overlap with spiradenoma and cylindroma.1 It may manifest as a solitary lesion or multiple lesions and can occur sporadically or in the context of a family history. Histologically, it must be distinguished from other intradermal basaloid neoplasms including conventional cylindroma and spiradenoma, dermal duct tumor, hidradenoma, and trichoblastoma.
When patients present with multiple cylindromas, spiradenomas, or spiradenocylindromas, physicians should consider genetic testing and review of the family history to assess for cylindromatosis gene mutations or Brooke-Spiegler syndrome. Biopsy and histologic examination are important because malignant tumors can evolve from pre-existing spiradenocylindromas, cylindromas, and spiradenomas,2 with an increased risk in patients with Brooke-Spiegler syndrome.1 Our patient declined further genetic workup but continues to follow up with dermatology for monitoring of lesions.
Dermal duct tumors are morphologic variants of poromas that are derived from sweat gland lineage and usually manifest as solitary dome-shaped papules, plaques, or nodules most often seen on acral surfaces as well as the head and neck.3 Clinically, they may be indistinguishable from spiradenocylindromas and require biopsy for histologic evaluation. They can be distinguished from spiradenocylindroma by the presence of small dermal nodules composed of cuboidal cells with ample pink cytoplasm and cuticle-lined ducts (Figure 1).
Trichoblastomas typically are deep-seated basaloid follicular neoplasms on the scalp with papillary mesenchyme resembling the normal fibrous sheath of the hair follicle, often replete with papillary mesenchymal bodies (Figure 2). There generally are no retraction spaces between its basaloid nests and the surrounding stroma, which is unlikely to contain mucin relative to basal cell carcinoma (BCC).4,5
Adenoid cystic carcinoma is a rare salivary gland tumor that can metastasize to the skin and rarely arises as a primary skin adnexal tumor. It manifests as a slowgrowing mass that can be tender to palpation.6 Histologic examination shows dermal islands with cribriform blue and pink spaces. Compared to BCC, adenoid cystic carcinoma cells are enlarged and epithelioid with relatively scarce cytoplasm (Figure 3).6,7 Adenoid cystic carcinoma can show variable growth patterns including infiltrative nests and trabeculae. Perineural invasion is common, and there is a high risk for local recurrence.7 First-line therapy usually is surgical, and postoperative radiotherapy may be required.6,7
Nodular BCC commonly manifests as an enlarging nonhealing lesion on sun-exposed skin and has many subtypes, typically with arborizing telangiectases on dermoscopy. Histopathologic examination of nodular BCC reveals a nest of basaloid follicular germinative cells in the dermis with peripheral palisading and a fibromyxoid stroma (Figure 4).8 Patients with Brooke-Spiegler syndrome are at increased risk for nodular BCC, which may be clinically indistinguishable from spiradenoma, cylindroma, and spiradenocylindroma, necessitating histologic assessment.
- Facchini V, Colangeli W, Bozza F, et al. A rare histopathological spiradenocylindroma: a case report. Clin Ter. 2022;173:292-294. doi:10.7417/ CT.2022.2433
- Kazakov DV. Brooke-Spiegler syndrome and phenotypic variants: an update [published online March 14, 2016]. Head Neck Pathol. 2016;10:125-30. doi:10.1007/s12105-016-0705-x
- Miller AC, Adjei S, Temiz LA, et al. Dermal duct tumor: a diagnostic dilemma. Dermatopathology (Basel). 2022;9:36-47. doi:10.3390/dermatopathology9010007
- Elston DM. Pilar and sebaceous neoplasms. In: Elston DM, Ferringer T, Ko C, et al. Dermatopathology. 3rd ed. Elsevier; 2018:71-85.
- McCalmont TH, Pincus LB. Adnexal neoplasms. In: Bolognia J, Schaffer J, Cerroni, L. Dermatology. 4th ed. Elsevier; 2017:1930-1953.
- Coca-Pelaz A, Rodrigo JP, Bradley PJ, et al. Adenoid cystic carcinoma of the head and neck—an update [published online May 2, 2015]. Oral Oncol. 2015;51:652-661. doi:10.1016/j.oraloncology.2015.04.005
- Tonev ID, Pirgova YS, Conev NV. Primary adenoid cystic carcinoma of the skin with multiple local recurrences. Case Rep Oncol. 2015;8:251- 255. doi:10.1159/000431082
- Cameron MC, Lee E, Hibler BP, et al. Basal cell carcinoma: epidemiology; pathophysiology; clinical and histological subtypes; and disease associations [published online May 18, 2018]. J Am Acad Dermatol. 2019;80:303-317. doi:10.1016/j.jaad.2018.03.060
- Facchini V, Colangeli W, Bozza F, et al. A rare histopathological spiradenocylindroma: a case report. Clin Ter. 2022;173:292-294. doi:10.7417/ CT.2022.2433
- Kazakov DV. Brooke-Spiegler syndrome and phenotypic variants: an update [published online March 14, 2016]. Head Neck Pathol. 2016;10:125-30. doi:10.1007/s12105-016-0705-x
- Miller AC, Adjei S, Temiz LA, et al. Dermal duct tumor: a diagnostic dilemma. Dermatopathology (Basel). 2022;9:36-47. doi:10.3390/dermatopathology9010007
- Elston DM. Pilar and sebaceous neoplasms. In: Elston DM, Ferringer T, Ko C, et al. Dermatopathology. 3rd ed. Elsevier; 2018:71-85.
- McCalmont TH, Pincus LB. Adnexal neoplasms. In: Bolognia J, Schaffer J, Cerroni, L. Dermatology. 4th ed. Elsevier; 2017:1930-1953.
- Coca-Pelaz A, Rodrigo JP, Bradley PJ, et al. Adenoid cystic carcinoma of the head and neck—an update [published online May 2, 2015]. Oral Oncol. 2015;51:652-661. doi:10.1016/j.oraloncology.2015.04.005
- Tonev ID, Pirgova YS, Conev NV. Primary adenoid cystic carcinoma of the skin with multiple local recurrences. Case Rep Oncol. 2015;8:251- 255. doi:10.1159/000431082
- Cameron MC, Lee E, Hibler BP, et al. Basal cell carcinoma: epidemiology; pathophysiology; clinical and histological subtypes; and disease associations [published online May 18, 2018]. J Am Acad Dermatol. 2019;80:303-317. doi:10.1016/j.jaad.2018.03.060
A 62-year-old man with a history of cylindromas presented to our clinic with multiple asymptomatic, 3- to 4-mm, nonmobile, dome-shaped, telangiectatic, pink papules over the parietal and vertex scalp that had been present for more than 10 years without change. Several family members had similar lesions that had not been evaluated by a physician, and there had been no genetic evaluation. Shave biopsies of several lesions were performed.
