Primary carcinosarcoma of the helix of the ear.

Abstract

We report our histologic and immunohistochemical findings in a rare case of cutaneous carcinosarcoma involving the helix of the ear. The tumor exhibited cellular features of both basal cell and squamous cell carcinoma and a malignant mesenchymal component that was consistent with malignant fibrous histiocytoma. The epithelial component exhibited a positive immunohistochemical reaction to cytokeratin and a negative reaction to vimentin, whereas the mesenchymal component showed a positive immunohistochemical reaction to vimentin and a negative reaction to cytokeratin. To the best of our knowledge, this is only the third reported case of a carcinosarcoma of the ear and the second case in which it developed on the helix.

Introduction

Carcinosarcoma is a rare tumor in which both elements--the epithelial and mesenchymal components--are malignant. This tumor has been reported to occur at various sites, including the adrenal gland, colon, endometrium, lung, oral cavity, and prostate; at each of these sites, the cancer is associated with metastasis and a poor outcome.

Primary cutaneous involvement is extremely rare, as we found only 20 cases that were previously reported in the literature (table). (1-15) All of these cutaneous carcinosarcomas had developed in patients who were older than 40 years, and most of them behaved in a manner that was clinically less aggressive than the progression of carcinosarcomas at other sites. To the best of our knowledge, only 2 cases of carcinosarcoma of the ear (9,14) and 1 case of carcinosarcoma of the ear helix (14) have been previously reported. We report a new case of carcinosarcoma of the helix.

Case report

A 73-year-old man presented with an enlarging, semifirm, 3.5 x 2.5-cm lesion that had arisen on the helix of the left ear and extended into the concha (figure 1). The mass, which had begun to develop 1 year earlier, was minimally mobile, and the overlying skin was not ulcerated. The clinical picture suggested a squamous cell carcinoma, The patient denied any other significant medical problems. External examination and a clinical workup did not reveal any enlarged lymph nodes or distant metastasis.

[FIGURE 1 OMITTED]

Wide excision of the ear was performed. Intraoperatively, frozen sections were obtained in an effort to ensure negative surgical margins. Gross pathologic examination identified the mass as a 3.5 x 2.5 x 2.5-cm polypoid tumor. On cross-section analysis, the tumor was firm and 0.5 cm from the deep margin.

Microscopic examination revealed that the tumor had originated in squamous epithelium. Large aggregates of basaloid epithelium were consistent with the histology of a basal cell carcinoma (figure 2, A). The tumor also exhibited some degree of squamous differentiation that was suggestive of a squamous cell carcinoma (figure 2, B). Large cystic spaces, areas of hyalinization, and focal necrosis were identified. The tumor also exhibited peripheral palisading of nuclei, some ductal differentiation, and the presence of closely associated hyalinized stroma, suggesting focal sweat gland differentiation. Mitotic figures were easily observed throughout the tumor's epithelial component. A large portion of the tumor contained a highly pleomorphic spindle-cell stroma with numerous multinucleated and bizarre cells and numerous mitoses. Histologically, the tumor's mesenchymal component was most consistent with a malignant fibrous histiocytoma (figure 2, C).

[FIGURE 2 OMITTED]

On immunoperoxidase staining, the epithelial component was positive for cytokeratin and p63 and negative for vimentin and epithelial membrane antigen (EMA) (figure 3, A). The mesenchymal component was positive for vimentin and CD 10, focally positive for CD68 and factor XIII, and negative for cytokeratin, S-100, caldesmon, and smooth-muscle actin (figure 3, B). Both components demonstrated a high proliferation index with the Ki-67 proliferation marker. Based on the histomorphologic findings, we established a diagnosis of carcinosarcoma.

[FIGURE 3 OMITTED]

At the 6-month postoperative follow-up, the patient manifested no evidence of distant metastasis or recurrence.

Discussion

Cutaneous carcinosarcomais a biphasic malignant tumor made up of both epithelial and mesenchymal components, (2,5,6,11,13,14) Our review of the literature revealed a lack of consensus regarding terminology, as this entity is variously referred to as spindle-cell carcinoma, sarcomatoid carcinoma, metaplastic carcinoma, and pseudosarcoma. (14)

The prognosis for cutaneous carcinosarcoma appears to be better than the prognosis for its visceral counterparts. H In the 20 previously reported cases of cutaneous carcinosarcoma, metastasis developed in only 4 patients (table). (2,4,6,10) On the other hand, most patients with carcinosarcomas in other organs develop metastasis. (11) No distant metastasis or local recurrence occurred in the 2 previously reported cases of cutaneous carcinosarcoma of the external ear following complete local excision. (9,14)

Among the 20 previously reported cases of cutaneous carcinosarcoma, the most common epithelial component was squamous cell carcinoma, followed by basal cell carcinoma (table). The most common mesenchymal component was osteosarcoma, followed by chondrosarcoma and malignant fibrous histiocytoma.

The histogenesis of these tumors remains controversial. Several theories have been suggested, including the collision tumor theory, the conversion theory, and the clonal evolution theory. According to the collision theory, two simultaneous and completely separate neoplasms arise at the same time at the same site. (1) The conversion theory, probably the most widely accepted, suggests either a sarcomatous transformation of a preexisting carcinoma (4-8,12,16) or an epidermal transformation induced by a dermal fibroblastic tumor. (17) Proponents of the clonal theory, also called the divergence theory, hypothesize that a single totipotential stem cell differentiates into separate epithelial and mesenchymal components. (10,11,14,17)

In our patient, the immunophenotypes of the epithelial and mesenchymal tumor components were different. Although this tumor was histopathologically atypical and its proliferation index was high, the potential for metastasis or recurrence following complete surgical removal appeared to be low.

Vladimir Vincek, MD, PhD; Marjan Mirzabeigi, MD; Brian S. Jewett, MD; W. Jarrard Goodwin, MD

Reprint requests: Vladimir Vincek, MD, Department of Pathology, Jackson Memorial Medical Center, University of Miami, Holtz Center, Room 2042, 1611 N.W. 12th Ave., Miami, FL 33136. Phone: [phone]; fax: [phone]: e-mail: [e-mail]

References

(1.) Dawson EK. Carcinosarcoma of the skin. J R Coil Surg 1972;17: 242-7.

(2.) Quay SC, Harrist TJ, Mihm MC Jr. Carcinosarcoma of the skin. Case report and review. J Cutan Pathol 1981;8:241-6.

(3.) Iakovides J, Delides GS. Carcinosarcomas of the skin--Report of two cases. Arch Geschwulstforsch 1988;58:461-4.

(4.) Scott A, Metcalf JS. Cutaneous malignant mixed tumor. Report of a case and review of the literature. Am J Dermatopathol 1988; 10: 335-42.

(5.) Tschen JA, Goldberg LH, McGavran MH. Carcinosarcoma of the skin. J Cutan Pathol 1988;15:31-5.

(6.) McKee PH, Fletcher CD, Stavrinos P, Pambakian H. Carcinosarcoma arising in eccrine spiradenoma. A clinicopathologic and immunohistochemical study of two cases. Am J Dermatopathol 1990; 12:335-43.

(7.) Parham DM, Morton K, Robertson AJ, Philip WD. The changing phenotypic appearance of a malignant vulval neoplasm containing both carcinomatous and sarcomatous elements. Histopathology 1991;19:263-8.

(8.) Izaki S, Hirai A, Yoshizawa Y, et al. Carcinosarcoma of the skin: Immunohistochemical and electron microscopic observations. J Cutan Pathol 1993;20:272-8.

(9.) Leen EJ, Saunders MP, Vollum DI, Keen CE. Carcinosarcoma of the skin. Histopathology 1995;26:367-71.

(10.) Biernat W, Kordek R, Liberski PP, Wozniak L. Carcinosarcoma of the skin. Case report and literature review. Am J Dermatopathol 1996;18:614-19.

(11.)Patel NK, McKee PH, Smith NP, Fletcher CD. Primary metaplastic carcinoma (carcinosarcoma) of the skin. A clinicopathologic study of four cases and review of the literature. Am J Dermatopathol 1997; 19:363-72.

(12.) Rios-Martin JJ, Parra-Martin JA, Gomez-Pascual A, et al. Sarcomatoid carcinoma of the skin: Report of a case. J Dermatol 1998;25:314-21.

(13.) Brown TJ, Tschen JA. Primary carcinosarcoma of the skin: Report of a case and review of the literature. Dermatol Surg 1999;25: 498-500.

(14.) Gomez-Espejo C, Herrera-Sabal A, Rios-Martin JJ, Camacho-Martinez F. Basal cell carcinoma with sarcomatoid features (sarcomatoid carcinoma): Report of a case and review of the literature. J Dermatol 2003;30:543-9.

(15.) Inaloz HS, Ayyalaraju RS, Holt PJ, Laidler P. Acase of sarcomatoid carcinoma of the skin. J Eur Acad Dermatol Venereol 2003;17: 59-61.

(16.) Pinkus H, Mehregan AH. A Guide to Dermatohistopathology. 3rd ed. New York: Appleton-Century-Crofts, 1981:457.

(17.) Torenbeek R, Hermsen MA, Meijer GA, et al. Analysis by comparative genomic hybridization of epithelial and spindle cell components in sarcomatoid carcinoma and carcinosarcoma: Histogenetic aspects. J Pathol 1999;189:338-43.

From the Department of Pathology (Dr. Vincek and Dr. Mirzabeigi) and the Department of Otolaryngology (Dr. Jewett and Dr. Goodwin). University of Miami School of Medicine.

Table. Summary of clinical and histological
features in the 21 reported cases (including
ours) of primary cutaneous carcinosarcoma

                                        Epithelial
Author                      Site         component

Dawson,                    Chest            BCC
(1) 1972

Quay et al,                Axilla         SCC/BCC
(2) 1981

Iakovides and             Forearm           SCC
Delides,                  Forearm           SCC
(3) 1988

Scott and                   Neck            SCC
Metcalf,
(4) 1988

Tschen et                 Forehead          BCC
al, (5)
1988

McKee et al,            Iliac crest     Spiradenoma
(6) 1990                    Arm         Spiradenoma

Parham et al,              Vulva            SCC
(7) 1991

Izaki et al,                Back            BCC
(8) 1993

Leen et al,                Tragus           BCC
(9) 1995

Biernat et al,              Arm             SCC
(10) 1996

Patel et al,               Eyelid           SCC
(11) 1997                  Scalp            SCC
                           Scalp            SCC
                            Nose            SCC

Rios-Martin et al,       Suprapubic         BCC
(12) 1998                   area

Brown and Tschen,           Arm             BCC
(13) 1999

Gomez-Espejo et al,      Ear helix          BCC
(14) 2003

Inaloz et al,             Forearm           BCC
(15) 2003

Vincek et al,            Ear helix        SCC/BCC
* 2005

                        Mesenchymal
Author                   component      Metastasis

Dawson,                    F-sar,           No
(1) 1972                 C-sar, SC

Quay et al,            C-sar, O-sar,        Yes
(2) 1981                 SC, F-sar

Iakovides and               MFH             No
Delides,                    MFH             No
(3) 1988

Scott and                  C-sar            Yes
Metcalf,
(4) 1988

Tschen et                  O-sar            No
al, (5)
1988

McKee et al,            O-sar, R-sar        Yes
(6) 1990                   O-sar            No

Parham et al,              O-sar            No
(7) 1991

Izaki et al,              Spindle           No
(8) 1993                 cell tumor

Leen et al,                O-sar            No
(9) 1995

Biernat et al,          Pleomorphic         Yes
(10) 1996                 sarcoma

Patel et al,               O-sar            No
(11) 1997               L-sar, R-sar        No
                        O-sar, C-sar        No
                           O-sar            No

Rios-Martin et al,          MFH             No
(12) 1998

Brown and Tschen,          F-sar            No
(13) 1999

Gomez-Espejo et al,         MFH             No
(14) 2003

Inaloz et al,            Atypical           No
(15) 2003                 fibrous
                          xanthoma

Vincek et al,               MFH             No
* 2005

* Present study.

Key: BCC = basal cell carcinoma; F-sar = fibrosarcoma;
C-sar = chondrosarcoma; SC = synovial carcinoma;
SCC =squamous cell carcinoma; O-sar = osteosarcoma;
MFH = malignant fibrous histiocytoma;
R-sar = rhabdoinyosarcoma; L-sar = leiomyosarcoma.