Histopathology 2014, 65, 658–666. DOI: 10.1111/his.12448
Spiradenocarcinoma, cylindrocarcinoma and spiradenocylindrocarcinoma: a clinicopathological study of nine cases Bo Dai,1,2 Yun-Yi Kong,2,3 Xu Cai,2,3 Xu-Xia Shen2,3 & Jin-Cheng Kong4 1
Department of Urology, Fudan University Shanghai Cancer Centre, Shanghai, China, 2Department of Oncology, Shanghai Medical College, Fudan University, Shanghai, China, 3Department of Pathology, Fudan University Shanghai Cancer Centre, Shanghai, China, and 4Department of Pathology, First People’s Hospital, Shanghai Jiaotong University, Shanghai, China
Date of submission 24 March 2014 Accepted for publication 29 April 2014 Published online Article Accepted 5 May 2014
Dai B, Kong Y-Y, Cai X, Shen X-X, Kong J-C (2014) Histopathology 65, 658–666
Spiradenocarcinoma, cylindrocarcinoma and spiradenocylindrocarcinoma: a clinicopathological study of nine cases Aims: To elucidate diagnostic criteria for spiradenocarcinoma, cylindrocarcinoma and spiradenocylindrocarcinoma, and to emphasize correlations between clinical behaviour and variable morphological patterns. Methods and results: We investigated the clinicopathological and immunophenotypic features of nine cases. There were five men and four women, with ages ranging from 58 years to 82 years. The tumour size varied from 10 mm to 50 mm. The head and neck were most commonly involved. Three cases of spiradenocarcinoma and three cases of cylindrocarcinoma showed a salivary gland-type basal cell adenocarcinoma-like pattern, low-grade (BCAC-LG) and/or high grade (BCAC-HG). The remaining three cases of spiradeno-
carcinoma showed adenocarcinoma in situ, with invasive adenocarcinoma being seen in one of these cases. PAS staining revealed loss of the PAS-positive hyaline sheath in malignant zones of cylindrocarcinoma. p53 staining was variably positive in the malignant components of all cases. Follow-up was available for all patients, ranging from 5 months to 107 months. Two patients died of disease, one experienced recurrence, and one died of an unrelated cause. Conclusions: Patients with BCAC-LG have a better prognosis. BCAC-HG is more likely to be found in cylindrocarcinoma, and its clinical behaviour seems to be more aggressive. Close follow-up for early detection of recurrence and metastases is strongly recommended.
Keywords: cylindrocarcinoma, immunohistochemistry, prognosis, spiradenocarcinoma
Introduction Spiradenoma, cylindroma and spiradenocylindroma are benign adnexal neoplasms of the skin, and constitute a spectrum of related lesions with similar histological and phenotypic features.1,2 Spiradenoma and cylindroma represent two extreme poles of the morphological spectrum, whereas spiradenocylindroma Address for correspondence: Dr Y-Y Kong, MD, PhD, Department of Pathology, Fudan University Shanghai Cancer Centre, 270 Dong’an Road, Shanghai 200032, China. e-mail: [email protected] © 2014 John Wiley & Sons Ltd.
occupies an intermediate position as a hybrid tumour. These lesions frequently occur as sporadic solitary neoplasms, but may also be components of Brooke– Spiegler syndrome (BBS), a disease inherited in an autosomal dominant fashion. The malignant counterparts, termed spiradenocarcinoma, cylindrocarcinoma, and spiradenocylindrocarcinoma, are extremely rare. Since the first case of cylindrocarcinoma was described by Wiedmann et al.3 in 1929, and the first two cases of spiradenocarcinoma were described by Dabska et al.4 in 1972, ~100 cases of spiradenocarcinoma, cylindrocarcinoma, and spiradenocylindrocarcinoma
Spiradenocarcinoma and cylindrocarcinoma
have been reported.5 However, on the basis of the illustrations provided, not all of those cases may be accepted as such.6 Furthermore, most have been published as case reports, with the only two series, of 24 cases and 12 cases respectively, described by Kazakov et al.5 and Granter et al.7 Herein, we present our clinical, histological and immunohistochemical study of nine cases of spiradenocarcinoma, cylindrocarcinoma, or spiradenocylindrocarcinoma, to further delineate diagnostic criteria for this rare tumour, and to emphasize correlations between clinical behaviour and prognosis with variable morphological patterns.
Materials and methods A search for spiradenocarcinoma, cylindrocarcinoma and spiradenocylindrocarcinoma or malignant spiradenoma, malignant cylindroma and malignant spiradenocylindroma was performed in the surgical pathology and consultation files of the Department of Pathology, Fudan University Shanghai Cancer Centre from 2004 to 2013. According to the morphological definition in the WHO classification8 and previous literature, a residuum of an unequivocally benign neoplasm (spiradenoma, cylindroma, or spiradenocylindroma) was required in the same case. Nine cases met the above selection criteria, and form the basis of this study. Available data, including clinical presentation, therapeutic regimen, and follow-up information, were collected. Informed consent was obtained from each patient, and the study was approved by our institutional ethics committee. Resection specimens of nine cases were all formalin-fixed, routinely processed, and paraffin-embedded. Sections were stained with haematoxylin and eosin (H&E) and periodic acid–Schiff (PAS). Immunohistochemical studies with the Envision method were performed using antibodies against p63 (4A4; Roche, Tucson, USA), p53 (DO-7; Dako, Glostrup, Denmark), Ki67 (MIB-1; Dako), GCDFP-15 (23A3; Dako), and CK7 (OV-TL; Dako).
Results CLINICAL DATA
The basic clinical information is summarized in Table 1. All nine cases were sporadic neoplasms. There were five men and four women, with the age at diagnosis ranging from 58 years to 82 years (mean, 67 years; median, 66 years). All patients presented with a solitary nodule or mass, varying from 10 mm © 2014 John Wiley & Sons Ltd, Histopathology, 65, 658–666.
659
to 50 mm in maximum dimension (Figure 1A,B). The lesions had been present for 1 year to 58 years before surgical removal (average duration, 15 years). Seven patients had a history of recent rapid growth. Three patients complained of tenderness. Recent colour change and ulceration of the skin lesion were noted in single cases. The head (including the face and scalp) and neck were the most frequently involved sites (n = 6), followed by the trunk (n = 2) and lower extremities (n = 1). Skin lesions were resected in all nine patients. In addition, right inguinal lymphadenectomy was performed simultaneously in one patient, but no regional metastasis was found in the resected lymph nodes. Adjuvant radiotherapy after surgical excision was performed in another patient. Follow-up was available for all nine patients, ranging from 5 months to 107 months, with a mean of ~44 months. Five patients were alive without evidence of disease, two died of disease, one experienced local recurrence, and one died of an unrelated cause. HISTOLOGY AND IMMUNOHISTOCHEMISTRY
The residuum of a benign neoplasm was present in all cases: spiradenoma in six, and cylindroma in three. A typical spiradenocylindroma component was not found in any cases. Transition between benign and malignant components was gradual. Microscopically, the tumours were located either intradermally (three cases) or intradermally with extension into subcutaneous tissue (six cases). In case 3, a muscle of facial expression was focally involved. At scanning magnification, five cases showed a lobular growth pattern with pushing borders, and the other four cases showed an infiltrative pattern with fibrous stromal reaction and scattered lymphocytes around. Stromal calcification was found occasionally in two cases. Ectatic blood vessels with ‘blood lakes’ and/or dilated lymphatic spaces were found in the stroma of six cases. The mitotic rate in the malignant components of the nine cases was variable, ranging from two to 62 per 10 high-power fields. Neither lymphovascular nor perineural invasion was found in any case. According to the morphological pattern of malignant components proposed by Kazakov et al.,5 three cases of spiradenocarcinoma showed a salivary gland-type basal cell adenocarcinoma-like (BCAC-like) pattern: a combination of BCAC low grade (BCAC-LG) and high grade (BCAC-HG) in two cases; and BCAC-LG only in one case. This pattern was composed of basaloid cells arranged in cords, trabeculae, nodules, or sheets. The typical dual cell population and scattered lymphocytes
660
B Dai et al.
Table 1. Summary of clinical information in nine cases of spiradenocarcinoma or cylindrocarcinoma
Case
Sex/age (years)
Derivation
Clinical features
Size (mm)
Location
Duration before diagnosis (years)
1
F/72
Spiradenoma
Solitary nodule
10
Face
1
Excision
72 months: DUC
2
F/69
Cylindroma
An exophytic dome-shaped mass with mild tenderness
40
Neck
20
Excision + radiotherapy
60 months: DOD
3
F/60
Spiradenoma
Solitary tumour
40
Face
3
Excision
107 months: recurrence
4
M/60
Spiradenoma
Solitary tumour
45
Chest
20
Excision
75 months: NED
5
M/82
Cylindroma
Solitary tumour with ulceration
40
Face
8
Excision
35 months: DOD (bilateral lung metastases)
6
M/63
Spiradenoma
Solitary nodule with tenderness
20
Face
58
Excision
16 months: NED
7
M/69
Spiradenoma
Solitary nodule with tenderness
20
Right thigh
10
Excision + right inguinal lymphadenectomy
15 months: NED
8
F/58
Cylindroma
Solitary nodule
15
Scalp
4
Excision
9 months: NED
9
M/66
Spiradenoma
Solitary tumour with colour change
50
Abdomen
10
Excision
5 months: NED
Treatment
Follow-up and outcome
DOD, died of disease; DUC, died from unrelated cause; NED, no evidence of disease.
were lost. Basaloid cells in BCAC-LG pattern were small to medium-sized, with mild to moderate nuclear pleomorphism and variable nuclear hyperchromasia. Nucleoli were small or absent (Figure 2A,B). However, in the BCAC-HG pattern, medium to large pleomorphic basaloid cells often grew as confluent nodules and sheets in an infiltrative pattern. Tumour cells had vesicular nuclei containing conspicuous nucleoli (Figure 2C,D). Also, focal to extensive tumour necrosis was frequently found. The remaining three cases of spiradenocarcinoma showed adenocarcinoma in situ, which has a gradual transition with adjacent benign and atypical adenomatous components. This morphological pattern was typified by small or tubular well-formed glandular structures with preserved myoepithelial cells. Cuboidal to columnar luminal cells had pale to eosinophilic cytoplasm. In benign and atypical adenomatous areas, glandular luminal cells were bland and monomorphous or showed mild atypia, with few mitoses. However, intraluminal proliferation of cells with both
obvious cytological atypia and numerous mitoses was found in adenocarcinoma in situ (Figure 3A). In one of these three cases, besides the adenocarcinoma in situ component, glandular or alveolar structures devoid of a peripheral myoepithelial cell layer were also found, indicating high-grade invasive adenocarcinoma, not otherwise specified (IAC-NOS) (Figure 3B). Also, scattered psammoma-body-like calcification and necrosis were found within epithelial islands of invasive adenocarcinoma (Figure 3C). Both apocrine secretion and mucinous metaplasia were found in two cases (Figure 3D). In three cases of cylindrocarcinoma, a BCAC-HG pattern was found as the only malignant element in two cases, whereas a combination of BCAC-LG and BCAC-HG was observed in one case. Well-defined tubular structures were identified in two of these three cases (Figure 4A). Multiple foci of squamous differentiation, termed ‘squamous morulas’, were seen in two cases (Figure 4B). Clear cell change was found in one case with a BCAC-HG pattern. In all three © 2014 John Wiley & Sons Ltd, Histopathology, 65, 658–666.
Spiradenocarcinoma and cylindrocarcinoma
A
661
by tumour cells with apocrine differentiation in two cases of spiradenocarcinoma (Figure 5D). Expression of Ki67 varied from 15% to 50% in the malignant components, and was much higher than in the residual benign counterpart.
Discussion
B
Figure 1. A, Computed tomography reveals an exophytic mass on the root of the neck. B, Gross appearance shows a grey–white, relatively circumscribed tumour in the dermal–subcutaneous area, with multiple foci of haemorrhage or degenerative change.
cases, there was a variable component of eosinophilic basement membrane material forming thick rims around and within the epithelial islands (Figure 4C). PAS staining revealed partial or complete loss of the PAS-positive hyaline sheath in malignant zones, but well-preserved networks in the benign cylindroma areas with a jigsaw puzzle pattern (Figure 4D). Immunohistochemically, in benign or atypical adenomatous areas and adenocarcinoma in situ of spiradenocarcinomas, preserved myoepithelial cells were confirmed by p63 nuclear staining (Figure 5A). CK7 staining was partially positive in infiltrative epithelial cells and luminal cells (Figure 5B). p53 staining was variably positive in the malignant components of all nine cases (Figure 5C). GCDFP-15 was expressed © 2014 John Wiley & Sons Ltd, Histopathology, 65, 658–666.
As a diagnostic criterion for spiradenocarcinoma, cylindrocarcinoma, and spiradenocylindrocarcinoma, the presence of an identifiable residuum of a benign counterpart is a prerequisite. In the absence of a benign focus, the tumour can be confused microscopically with other skin malignancies and metastatic carcinomas. According to previous data, spiradenocarcinoma is the most commonly reported neoplasm, followed by cylindrocarcinoma, and spiradenocylindrocarcinoma is the rarest form. In our series, the residuum of a benign neoplasm was present in six cases of spiradenocarcinoma and three cases of cylindrocarcinoma. A spiradenocylindroma component was not found in any of our cases. Malignant transformation often occurs in a pre-existing benign tumour that has been present for many years, even several decades. As seen in our series, more than half of the patients had tumours for no
Spiradenocarcinoma, cylindrocarcinoma and spiradenocylindrocarcinoma: a clinicopathological study of nine cases Bo Dai,1,2 Yun-Yi Kong,2,3 Xu Cai,2,3 Xu-Xia Shen2,3 & Jin-Cheng Kong4 1
Department of Urology, Fudan University Shanghai Cancer Centre, Shanghai, China, 2Department of Oncology, Shanghai Medical College, Fudan University, Shanghai, China, 3Department of Pathology, Fudan University Shanghai Cancer Centre, Shanghai, China, and 4Department of Pathology, First People’s Hospital, Shanghai Jiaotong University, Shanghai, China
Date of submission 24 March 2014 Accepted for publication 29 April 2014 Published online Article Accepted 5 May 2014
Dai B, Kong Y-Y, Cai X, Shen X-X, Kong J-C (2014) Histopathology 65, 658–666
Spiradenocarcinoma, cylindrocarcinoma and spiradenocylindrocarcinoma: a clinicopathological study of nine cases Aims: To elucidate diagnostic criteria for spiradenocarcinoma, cylindrocarcinoma and spiradenocylindrocarcinoma, and to emphasize correlations between clinical behaviour and variable morphological patterns. Methods and results: We investigated the clinicopathological and immunophenotypic features of nine cases. There were five men and four women, with ages ranging from 58 years to 82 years. The tumour size varied from 10 mm to 50 mm. The head and neck were most commonly involved. Three cases of spiradenocarcinoma and three cases of cylindrocarcinoma showed a salivary gland-type basal cell adenocarcinoma-like pattern, low-grade (BCAC-LG) and/or high grade (BCAC-HG). The remaining three cases of spiradeno-
carcinoma showed adenocarcinoma in situ, with invasive adenocarcinoma being seen in one of these cases. PAS staining revealed loss of the PAS-positive hyaline sheath in malignant zones of cylindrocarcinoma. p53 staining was variably positive in the malignant components of all cases. Follow-up was available for all patients, ranging from 5 months to 107 months. Two patients died of disease, one experienced recurrence, and one died of an unrelated cause. Conclusions: Patients with BCAC-LG have a better prognosis. BCAC-HG is more likely to be found in cylindrocarcinoma, and its clinical behaviour seems to be more aggressive. Close follow-up for early detection of recurrence and metastases is strongly recommended.
Keywords: cylindrocarcinoma, immunohistochemistry, prognosis, spiradenocarcinoma
Introduction Spiradenoma, cylindroma and spiradenocylindroma are benign adnexal neoplasms of the skin, and constitute a spectrum of related lesions with similar histological and phenotypic features.1,2 Spiradenoma and cylindroma represent two extreme poles of the morphological spectrum, whereas spiradenocylindroma Address for correspondence: Dr Y-Y Kong, MD, PhD, Department of Pathology, Fudan University Shanghai Cancer Centre, 270 Dong’an Road, Shanghai 200032, China. e-mail: [email protected] © 2014 John Wiley & Sons Ltd.
occupies an intermediate position as a hybrid tumour. These lesions frequently occur as sporadic solitary neoplasms, but may also be components of Brooke– Spiegler syndrome (BBS), a disease inherited in an autosomal dominant fashion. The malignant counterparts, termed spiradenocarcinoma, cylindrocarcinoma, and spiradenocylindrocarcinoma, are extremely rare. Since the first case of cylindrocarcinoma was described by Wiedmann et al.3 in 1929, and the first two cases of spiradenocarcinoma were described by Dabska et al.4 in 1972, ~100 cases of spiradenocarcinoma, cylindrocarcinoma, and spiradenocylindrocarcinoma
Spiradenocarcinoma and cylindrocarcinoma
have been reported.5 However, on the basis of the illustrations provided, not all of those cases may be accepted as such.6 Furthermore, most have been published as case reports, with the only two series, of 24 cases and 12 cases respectively, described by Kazakov et al.5 and Granter et al.7 Herein, we present our clinical, histological and immunohistochemical study of nine cases of spiradenocarcinoma, cylindrocarcinoma, or spiradenocylindrocarcinoma, to further delineate diagnostic criteria for this rare tumour, and to emphasize correlations between clinical behaviour and prognosis with variable morphological patterns.
Materials and methods A search for spiradenocarcinoma, cylindrocarcinoma and spiradenocylindrocarcinoma or malignant spiradenoma, malignant cylindroma and malignant spiradenocylindroma was performed in the surgical pathology and consultation files of the Department of Pathology, Fudan University Shanghai Cancer Centre from 2004 to 2013. According to the morphological definition in the WHO classification8 and previous literature, a residuum of an unequivocally benign neoplasm (spiradenoma, cylindroma, or spiradenocylindroma) was required in the same case. Nine cases met the above selection criteria, and form the basis of this study. Available data, including clinical presentation, therapeutic regimen, and follow-up information, were collected. Informed consent was obtained from each patient, and the study was approved by our institutional ethics committee. Resection specimens of nine cases were all formalin-fixed, routinely processed, and paraffin-embedded. Sections were stained with haematoxylin and eosin (H&E) and periodic acid–Schiff (PAS). Immunohistochemical studies with the Envision method were performed using antibodies against p63 (4A4; Roche, Tucson, USA), p53 (DO-7; Dako, Glostrup, Denmark), Ki67 (MIB-1; Dako), GCDFP-15 (23A3; Dako), and CK7 (OV-TL; Dako).
Results CLINICAL DATA
The basic clinical information is summarized in Table 1. All nine cases were sporadic neoplasms. There were five men and four women, with the age at diagnosis ranging from 58 years to 82 years (mean, 67 years; median, 66 years). All patients presented with a solitary nodule or mass, varying from 10 mm © 2014 John Wiley & Sons Ltd, Histopathology, 65, 658–666.
659
to 50 mm in maximum dimension (Figure 1A,B). The lesions had been present for 1 year to 58 years before surgical removal (average duration, 15 years). Seven patients had a history of recent rapid growth. Three patients complained of tenderness. Recent colour change and ulceration of the skin lesion were noted in single cases. The head (including the face and scalp) and neck were the most frequently involved sites (n = 6), followed by the trunk (n = 2) and lower extremities (n = 1). Skin lesions were resected in all nine patients. In addition, right inguinal lymphadenectomy was performed simultaneously in one patient, but no regional metastasis was found in the resected lymph nodes. Adjuvant radiotherapy after surgical excision was performed in another patient. Follow-up was available for all nine patients, ranging from 5 months to 107 months, with a mean of ~44 months. Five patients were alive without evidence of disease, two died of disease, one experienced local recurrence, and one died of an unrelated cause. HISTOLOGY AND IMMUNOHISTOCHEMISTRY
The residuum of a benign neoplasm was present in all cases: spiradenoma in six, and cylindroma in three. A typical spiradenocylindroma component was not found in any cases. Transition between benign and malignant components was gradual. Microscopically, the tumours were located either intradermally (three cases) or intradermally with extension into subcutaneous tissue (six cases). In case 3, a muscle of facial expression was focally involved. At scanning magnification, five cases showed a lobular growth pattern with pushing borders, and the other four cases showed an infiltrative pattern with fibrous stromal reaction and scattered lymphocytes around. Stromal calcification was found occasionally in two cases. Ectatic blood vessels with ‘blood lakes’ and/or dilated lymphatic spaces were found in the stroma of six cases. The mitotic rate in the malignant components of the nine cases was variable, ranging from two to 62 per 10 high-power fields. Neither lymphovascular nor perineural invasion was found in any case. According to the morphological pattern of malignant components proposed by Kazakov et al.,5 three cases of spiradenocarcinoma showed a salivary gland-type basal cell adenocarcinoma-like (BCAC-like) pattern: a combination of BCAC low grade (BCAC-LG) and high grade (BCAC-HG) in two cases; and BCAC-LG only in one case. This pattern was composed of basaloid cells arranged in cords, trabeculae, nodules, or sheets. The typical dual cell population and scattered lymphocytes
660
B Dai et al.
Table 1. Summary of clinical information in nine cases of spiradenocarcinoma or cylindrocarcinoma
Case
Sex/age (years)
Derivation
Clinical features
Size (mm)
Location
Duration before diagnosis (years)
1
F/72
Spiradenoma
Solitary nodule
10
Face
1
Excision
72 months: DUC
2
F/69
Cylindroma
An exophytic dome-shaped mass with mild tenderness
40
Neck
20
Excision + radiotherapy
60 months: DOD
3
F/60
Spiradenoma
Solitary tumour
40
Face
3
Excision
107 months: recurrence
4
M/60
Spiradenoma
Solitary tumour
45
Chest
20
Excision
75 months: NED
5
M/82
Cylindroma
Solitary tumour with ulceration
40
Face
8
Excision
35 months: DOD (bilateral lung metastases)
6
M/63
Spiradenoma
Solitary nodule with tenderness
20
Face
58
Excision
16 months: NED
7
M/69
Spiradenoma
Solitary nodule with tenderness
20
Right thigh
10
Excision + right inguinal lymphadenectomy
15 months: NED
8
F/58
Cylindroma
Solitary nodule
15
Scalp
4
Excision
9 months: NED
9
M/66
Spiradenoma
Solitary tumour with colour change
50
Abdomen
10
Excision
5 months: NED
Treatment
Follow-up and outcome
DOD, died of disease; DUC, died from unrelated cause; NED, no evidence of disease.
were lost. Basaloid cells in BCAC-LG pattern were small to medium-sized, with mild to moderate nuclear pleomorphism and variable nuclear hyperchromasia. Nucleoli were small or absent (Figure 2A,B). However, in the BCAC-HG pattern, medium to large pleomorphic basaloid cells often grew as confluent nodules and sheets in an infiltrative pattern. Tumour cells had vesicular nuclei containing conspicuous nucleoli (Figure 2C,D). Also, focal to extensive tumour necrosis was frequently found. The remaining three cases of spiradenocarcinoma showed adenocarcinoma in situ, which has a gradual transition with adjacent benign and atypical adenomatous components. This morphological pattern was typified by small or tubular well-formed glandular structures with preserved myoepithelial cells. Cuboidal to columnar luminal cells had pale to eosinophilic cytoplasm. In benign and atypical adenomatous areas, glandular luminal cells were bland and monomorphous or showed mild atypia, with few mitoses. However, intraluminal proliferation of cells with both
obvious cytological atypia and numerous mitoses was found in adenocarcinoma in situ (Figure 3A). In one of these three cases, besides the adenocarcinoma in situ component, glandular or alveolar structures devoid of a peripheral myoepithelial cell layer were also found, indicating high-grade invasive adenocarcinoma, not otherwise specified (IAC-NOS) (Figure 3B). Also, scattered psammoma-body-like calcification and necrosis were found within epithelial islands of invasive adenocarcinoma (Figure 3C). Both apocrine secretion and mucinous metaplasia were found in two cases (Figure 3D). In three cases of cylindrocarcinoma, a BCAC-HG pattern was found as the only malignant element in two cases, whereas a combination of BCAC-LG and BCAC-HG was observed in one case. Well-defined tubular structures were identified in two of these three cases (Figure 4A). Multiple foci of squamous differentiation, termed ‘squamous morulas’, were seen in two cases (Figure 4B). Clear cell change was found in one case with a BCAC-HG pattern. In all three © 2014 John Wiley & Sons Ltd, Histopathology, 65, 658–666.
Spiradenocarcinoma and cylindrocarcinoma
A
661
by tumour cells with apocrine differentiation in two cases of spiradenocarcinoma (Figure 5D). Expression of Ki67 varied from 15% to 50% in the malignant components, and was much higher than in the residual benign counterpart.
Discussion
B
Figure 1. A, Computed tomography reveals an exophytic mass on the root of the neck. B, Gross appearance shows a grey–white, relatively circumscribed tumour in the dermal–subcutaneous area, with multiple foci of haemorrhage or degenerative change.
cases, there was a variable component of eosinophilic basement membrane material forming thick rims around and within the epithelial islands (Figure 4C). PAS staining revealed partial or complete loss of the PAS-positive hyaline sheath in malignant zones, but well-preserved networks in the benign cylindroma areas with a jigsaw puzzle pattern (Figure 4D). Immunohistochemically, in benign or atypical adenomatous areas and adenocarcinoma in situ of spiradenocarcinomas, preserved myoepithelial cells were confirmed by p63 nuclear staining (Figure 5A). CK7 staining was partially positive in infiltrative epithelial cells and luminal cells (Figure 5B). p53 staining was variably positive in the malignant components of all nine cases (Figure 5C). GCDFP-15 was expressed © 2014 John Wiley & Sons Ltd, Histopathology, 65, 658–666.
As a diagnostic criterion for spiradenocarcinoma, cylindrocarcinoma, and spiradenocylindrocarcinoma, the presence of an identifiable residuum of a benign counterpart is a prerequisite. In the absence of a benign focus, the tumour can be confused microscopically with other skin malignancies and metastatic carcinomas. According to previous data, spiradenocarcinoma is the most commonly reported neoplasm, followed by cylindrocarcinoma, and spiradenocylindrocarcinoma is the rarest form. In our series, the residuum of a benign neoplasm was present in six cases of spiradenocarcinoma and three cases of cylindrocarcinoma. A spiradenocylindroma component was not found in any of our cases. Malignant transformation often occurs in a pre-existing benign tumour that has been present for many years, even several decades. As seen in our series, more than half of the patients had tumours for no
