Sebaceous Differentiation in Poroid Neoplasms: Report of 11 Cases, Including a Case of Metaplastic Carcinoma Associated with Apocrine Poroma (Sarcomatoid Apocrine Porocarcinoma)

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Kazakov, D V; Kutzner, H; Spagnolo, D V; Kempf, W; Zelger, B; Mukensnabl, P; Michal, M (2008). Sebaceous differentiation in poroid neoplasms: report of 11 cases, including a case of metaplastic carcinoma associated with apocrine poroma (sarcomatoid apocrine porocarcinoma). American Journal of Dermatopathology, 30(1):21-26. Postprint available at: http://www.zora.uzh.ch

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Originally published at: American Journal of Dermatopathology 2008, 30(1):21-26. Winterthurerstr. 190 CH-8057 Zurich http://www.zora.uzh.ch

Year: 2008

Sebaceous differentiation in poroid neoplasms: report of 11 cases, including a case of metaplastic carcinoma associated with apocrine poroma (sarcomatoid apocrine porocarcinoma)

Kazakov, D V; Kutzner, H; Spagnolo, D V; Kempf, W; Zelger, B; Mukensnabl, P; Michal, M

Posted at the Zurich Open Repository and Archive, University of Zurich. http://www.zora.uzh.ch

Originally published at: American Journal of Dermatopathology 2008, 30(1):21-26. Sebaceous differentiation in poroid neoplasms: report of 11 cases, including a case of metaplastic carcinoma associated with apocrine poroma (sarcomatoid apocrine porocarcinoma)

Abstract

We describe 11 poroid neoplasms with sebaceous differentiation, including a metaplastic (sarcomatoid) carcinoma arising in association with an apocrine poroma. Six lesions had the silhouette of a classical poroma, 3 of poroid hidradenoma and 1 of dermal duct tumor. In all cases, sebaceous differentiation was identified as clustered or solitary, mature sebocytes occurring mainly at the periphery of intradermal cellular aggregations, accompanied by sebaceous ducts. In one poroma, clusters of sebocytes were seen within intradermal aggregates and intraepidermally. In 1 of the 3 poroid hidradenomas, the eosinophilic cuticle lining the cyst was crenulated in foci associated with sebocytes. In none of the cases were there signs of follicular differentiation. One poroma, in addition to sebaceous differentiation, showed decapitation secretion in some ductular structures. The single carcinoma was an ulcerated oval to spindle cell neoplasm surrounded laterally by the residuum of a poroma containing groups of sebocytes. The epithelial islands of the poroma were prominently keratinized and blended gradually with the pleomorphic cells of the metaplastic carcinoma that immunohistochemically stained focally for cytokeratins and simultaneously showed strong vimentin expression. Our study supports previous findings that sebaceous differentiation can be identified not only in classical poroma but also in the related lesions known as dermal duct tumor and poroid hidradenoma. Occurrence of metaplastic carcinoma in association with apocrine poroma is a rare event which indicates the existence of a malignant counterpart of the latter entity, which can be descriptively referred to as "sarcomatoid apocrine porocarcinoma." ORIGINAL ARTICLE

Sebaceous Differentiation in Poroid Neoplasms: Report of 11 Cases, Including a Case of Metaplastic Carcinoma Associated With Apocrine Poroma (Sarcomatoid Apocrine Porocarcinoma) Dmitry V. Kazakov, MD, PhD,* Heinz Kutzner, MD,† Dominic V. Spagnolo, MMBS,‡ Werner Kempf, MD,§ Bernhard Zelger, MD, MSc,¶ Petr Mukensnabl, MD,* and Michal Michal, MD*

idroacanthoma simplex, poroma, dermal duct tumor, and Abstract: We describe 11 poroid neoplasms with sebaceous Hporoid hidradenoma are closely related, benign cutaneous differentiation, including a metaplastic (sarcomatoid) carcinoma adnexal neoplasms sharing a common cellular composition. arising in association with an apocrine poroma. Six lesions had the These tumors are composed of small, round, basophilic poroid silhouette of a classical poroma, 3 of poroid hidradenoma and 1 of cells and eosinophilic squamoid cells (cuticular cells). Small dermal duct tumor. In all cases, sebaceous differentiation was identified ductal structures may be present, mainly within the areas of as clustered or solitary, mature sebocytes occurring mainly at the cuticular cells. These four neoplasms differ in their silhouettes: periphery of intradermal cellular aggregations, accompanied by hidroacanthoma simplex is a horizontally oriented lesion sebaceous ducts. In one poroma, clusters of sebocytes were seen confined to the epidermis, consisting of discrete, round, within intradermal aggregates and intraepidermally. In 1 of the 3 poroid smooth-bordered cellular aggregates; poroma appears as hidradenomas, the eosinophilic cuticle lining the cyst was crenulated in interconnected epithelial cell aggregations emanating from foci associated with sebocytes. In none of the cases were there signs of the undersurface of the epidermis; in dermal duct tumor, follicular differentiation. One poroma, in addition to sebaceous differ- numerous well-circumscribed, neoplastic cellular aggregates entiation, showed decapitation secretion in some ductular structures. with conspicuous ducts are largely situated within the reticular The single carcinoma was an ulcerated oval to spindle cell neoplasm dermis; and poroid hidradenoma appears as a solid-cystic surrounded laterally by the residuum of a poroma containing groups of neoplasm confined entirely to the reticular dermis and with no sebocytes. The epithelial islands of the poroma were prominently kera- connection to the epidermis.1 ‘‘Hybrid’’ lesions featuring tinized and blended gradually with the pleomorphic cells of the meta- a combination of 2 or more of these poroid neoplasms have plastic carcinoma that immunohistochemically stained focally for been reported.2,3 cytokeratins and simultaneously showed strong vimentin expression. Traditionally, poroid neoplasms were considered eccrine. Our study supports previous findings that sebaceous differentiation can However, several reports documenting features of true apocrine be identified not only in classical poroma but also in the related lesions secretion or the presence of sebaceous and/or follicular dif- known as dermal duct tumor and poroid hidradenoma. Occurrence of ferentiation in poroma lead to the introduction of the term metaplastic carcinoma in association with apocrine poroma is a rare ‘‘apocrine poroma.’’4–13 Sebaceous differentiation has also event which indicates the existence of a malignant counterpart of the rarely been reported in poroid hidradenoma.14 The current latter entity, which can be descriptively referred to as ‘‘sarcomatoid World Health Organization classification includes poroma in the apocrine porocarcinoma.’’ category of benign adnexal tumors that may show either eccrine Key Words: apocrine poroma, carcinosarcoma, cutaneous adnexal or apocrine differentiation; in this classification the term tumors, metaplastic carcinoma, porocarcinoma, sarcomatoid apocrine ‘‘poroma’’ encompasses hidroacanthoma simplex, dermal duct porocarcinoma, sebaceous differentiation tumor, and the classical poroma, whereas poroid hidradenoma is considered together with nodular hidradenoma.15 (Am J Dermatopathol 2008;30:21–26) In this report we describe 11 poroid neoplasms with sebaceous differentiation. Included is a case of metaplastic (sarcomatoid) carcinoma arising in association with an apocrine From the *Sikl’s Department of Pathology, Charles University Medical Faculty poroma, which we have descriptively named ‘‘sarcomatoid Hospital, Pilsen, Czech Republic; †Dermatohistopathologische Gemein- apocrine porocarcinoma’’ (SAP). schaftspraxis, Friedrichshafen, Germany; ‡Division of Tissue Pathology, PathWest Laboratory Medicine WA, Nedlands, Western Australia, Australia; §Kempf und Pfaltz, Histologische Diagnostik, Zu¨rich, Switzerland; and {Clinical Department of Dermatology and Venereology, Innsbruck Medical MATERIALS AND METHODS University, Innsbruck, Austria. The 11 cases were found in the consultation or routine Reprints: Dmitry V. Kazakov, MD, PhD, Sikl’s Department of Pathology, Charles University Medical Faculty Hospital, Alej Svobody 80, 304 60 files of the authors. Sebaceous differentiation represented Pilsen, Czech Republic (e-mail: [email protected]). a minor but significant component of the 11 cases. They were Copyright Ó 2008 by Lippincott Williams & Wilkins identified among approximately 300 poroid lesions

Am J Dermatopathol Volume 30, Number 1, February 2008 21 Kazakov et al Am J Dermatopathol Volume 30, Number 1, February 2008 collectively seen by the authors, but this frequency of sebaceous differentiation is likely an overestimate of this occurrence owing to consultation case bias. The possibility that sebaceous areas represented preexisting sebaceous lobules entrapped by poroma was ruled out. Cases included in the study showed multiple foci of sebaceous differentiation, which were not associated with identifiable preexisting adnexa. These foci were extensive enough to ensure that definite distinction between de novo sebaceous differentiation and residual sebaceous elements from preexisting normal structures could be made confidently. Cases where entrapment could not be ruled out were excluded from the study. The essential clinical information was obtained from pathology and clinical records. Follow-up was not sought, except for the SAP. This study was based on light microscopy; only in SAP were immunohistochemical studies performed utilizing the following antibodies against cytokeratins (clone AE1:AE3, 1:50, DakoCytomation, Glostrup) and desmin (D33, 1:50, DakoCytomation, Glostrup).

RESULTS Clinical Data The main clinical information is summarized in Table 1. All tumors were surgically removed. The patient with the SAP (case 11) was without evidence of recurrence or metastatic disease 18 months after surgery. Histopathological Features Six of the benign lesions had the silhouette of a classical poroma, 3 of poroid hidradenoma and 1 of dermal duct tumor. In all cases, sebaceous differentiation was identiﬁed as clustered or solitary, mature sebocytes occurring mainly at the periphery of intradermal cellular aggregations, accompanied by sebaceous

TABLE 1. Main Clinicopathological Features Case Age/Sex Site Clinical Features Dominant Pattern Case 1 M/78 Upper lip Nodule, 6 3 6 mm Poroma Case 2 F/59 Right cheek Nodule. BCC? Poroma Case 3 F/46 Nose Nodule, 1 year Poroma duration. BCC? Wart? Case 4 F/29 Right calf Nodule. Lipoma? Poroma Case 5 F/78 Groin Solitary nodule Poroma Case 6 M/61 Periorbital Pyogenic Poroma skin granuloma? Case 7 M/38 Right thigh Nodule. Dermal duct tumor Histiocytoma? Case 8 F/43 Scalp Nodule Poroid hidradenoma Case 9 M/63 Hand Tumor Poroid hidradenoma Case 10 M/78 Scalp BCC Poroid hidradenoma FIGURE 1. Poroma with sebaceous differentiation. A, ulcerated Case 11 F/89 Buttock Ulcerated nodule Poroma with tumor with bulbous interconnected epithelial aggregates metaplastic emanating from the epidermis. B, the periphery of the tumor: (sarcomatoid) ductal structures, areas with sebaceous differentiation and carcinoma areas of necrosis en masse can be discerned. C, close-up of an BCC, basal cell carcinoma. area with sebaceous differentiation: clusters of sebocytes and sebaceous ducts can be identiﬁed.

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FIGURE 2. Poroma with sebaceous differentiation. A, B, C, clusters of sebaceous cells are evident within epidermis and within the dermal islands of neoplastic cells. ducts (Figs. 1–3). In one poroma, clusters of sebocytes were seen FIGURE 3. Poroid hidradenoma with sebaceous differentiation. within intradermal aggregates and intraepidermally (Fig. 2). In 1 A, solid-cystic lesion with no connection to the epidermis. of the 3 poroid hidradenomas, the eosinophilic cuticle lining the B, poroid cells, cuticular cells, and small ductal structures. cyst was crenulated in foci associated with sebocytes (Fig. 3). C, sebocytes in the vicinity of the cuticle which is somewhat The SAP was an ulcerated oval to spindle cell neoplasm crenulated and eosinophilic, similar to that seen in steatocys- surrounded laterally by the residuum of a poroma containing toma or isthmus of the hair follicle. q 2008 Lippincott Williams & Wilkins 23 Kazakov et al Am J Dermatopathol Volume 30, Number 1, February 2008

FIGURE 4. Metaplastic carcinoma associated with apocrine poroma (sarcomatoid apocrine porocarcinoma). A, B, ulcerated polypoid neoplasm with adjacent remnants of poroma on the right. C, sebaceous differentiation in the residuum of the poroma. D, transitional areas between the remnants of the poroma and metaplastic carcinoma, which focally assumes a spindle cell ap- pearance. E, atypical mitotic ﬁgures and prominent cellular pleomor- phism in the metaplastic carcinoma. F, G, spindled cells of the metaplastic carcinoma partially retain cytokera- tin expression (F, staining for AE1/ AE3) and simultaneously show strong vimentin positivity (G). groups of sebocytes. The epithelial islands of the poroma were differentiation, showed decapitation secretion in some ductular prominently keratinized and blended gradually with the pleo- structures. morphic cells of the metaplastic carcinoma that immunohistochemically stained focally for cytokeratins and simultaneously showed strong vimentin expression (Fig. 4). DISCUSSION In none of the cases were there signs of follicular Besides sebaceous carcinoma, sebaceoma, sebaceous differentiation. One poroma, in addition to sebaceous adenoma, and sebaceous cystic tumors, the 4 entities included

24 q 2008 Lippincott Williams & Wilkins Am J Dermatopathol Volume 30, Number 1, February 2008 Apocrine Poroma in the category of sebaceous tumors in the updated World 2. Kakinuma H, Miyamoto R, Iwasawa U, et al. Three subtypes of poroid Health Organization classification,15 foci of sebaceous neoplasia in a single lesion: eccrine poroma, hidroacanthoma simplex, and dermal duct tumor. Histologic, histochemical, and ultrastructural findings. differentiation can be encountered in a range of benign and Am J Dermatopathol. 1994;16:66–72. malignant adnexal tumors of the skin, including apocrine 3. Chen CC, Chang YT, Liu HN. Clinical and histological characteristics of mixed tumor, cylindroma, spiradenoma, tubular adenoma, poroid neoplasms: a study of 25 cases in Taiwan. Int J Dermatol. 2006;45: pilar tumor, microcystic adnexal carcinoma, and others.7,16–24 722–727. Conversely, rare sebaceous carcinomas and sebaceomas may 4. Hanau D, Grosshans E, Laplanche G. A complex poroma-like adnexal 25,26 adenoma. Am J Dermatopathol. 1984;6:567–572. show signs of apocrine differentiation. Some neoplasms 5. Zaim MT. Sebocrine adenoma. An adnexal adenoma with sebaceous and may show ‘‘divergent’’ differentiation reflecting all three apocrine poroma-like differentiation. Am J Dermatopathol. 1988;10: lineages of the folliculosebaceous apocrine unit, consistent 311–318. with the close embryological relationship of theses ele- 6. Harvell JD, Kerschmann RL, LeBoit PE. Eccrine or apocrine poroma? Six 24,27–31 poromas with divergent adnexal differentiation. Am J Dermatopathol. ments. Such dual or tripartite differentiation is often 1996;18:1–9. taken to support the apocrine nature of a neoplasm with ductal 7. Requena L, Kiryu H, Ackerman AB. Neoplasms With Apocrine differentiation and was used to support the concept of apocrine Differentiation. Philadelphia, PA: Lippincott-Raven; 1998. poroma.6,32 8. Gianotti R, Coggi A, Alessi E. Poral neoplasm with combined sebaceous Our study adds 11 further examples of poroid lesions and apocrine differentiation. Am J Dermatopathol. 1998;20:491–494. 9. Groben PA, Hitchcock MG, Leshin B, et al. Apocrine poroma: a distinctive with sebaceous differentiation to those previously reported. case in a patient with nevoid basal cell carcinoma syndrome. Am J The classical poroma pattern dominated in our small series, Dermatopathol. 1999;21:31–33. similar to other reports in the literature with the exception of 10. Lee NH, Lee SH, Ahn SK. Apocrine poroma with sebaceous occasional descriptions of sebaceous differentiation in poroid differentiation. Am J Dermatopathol. 2000;22:261–263. 6 11. Misago N, Mihara I, Ansai S, et al. Sebaceoma and related neoplasms with hidradenoma. Similar to the series of Harvell et al, none of sebaceous differentiation: a clinicopathologic study of 30 cases. 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