Skull Base 2005; 15(4): 275-280
DOI: 10.1055/s-2005-918885
CASE REPORT

Copyright © 2005 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001, USA.

Aggressive Syringomatous Carcinoma of the Orbit

Shingo Hasegawa1 , 6 , Eiji Kohmura2 , Akihiro Ichinose3 , Shinya Tahara3 , Atsushi Azumi4 , Chiho Ohbayashi5 , Ken-ichi Nibu1
  • 1Department of Otolaryngology-Head and Neck Surgery, Kobe University Hospital, Kobe, Japan
  • 2Department of Neurosurgery, Kobe University Hospital, Kobe, Japan
  • 3Department of Plastic Surgery, Kobe University Hospital, Kobe, Japan
  • 4Department of Ophthalmology, Kobe University Hospital, Kobe, Japan
  • 5Department of Pathology, Kobe University Hospital, Kobe, Japan
  • 6Department of Otolaryngology, Kohnan General Hospital, Kobe, Japan
Further Information

Publication History

Publication Date:
19 October 2005 (online)

ABSTRACT

A 68-year-old woman with an unusual tumor involving the right orbit presented with painful exophthalmos of the right eye. Excision biopsy of her right eyelid was performed. The specimen showed ductal differentiation with comma-like extensions identical to syringoma. Cellular atypia, an invasive growth pattern, and remarkable perineural invasion led to the diagnosis of syringomatous carcinoma. Magnetic resonance imaging showed that the tumor exhibited maxillary involvement, invading the supraorbital fissure and bone of the middle skull base. The patient underwent craniofacial resection and has been alive with no sign of recurrence for 2 years. This case suggests that wide excision of these tumors with a clear surgical margin using skull base surgical techniques may offer a good prognosis.

REFERENCES

  • 1 Goldstein D J, Barr R J, Santa Cruz D J. Microcystic adnexal carcinoma: a distinct clinicopathological entity.  Cancer. 1982;  50 566-572
  • 2 Sanchez Yus E, Requena Caballero L, Garcia Salazar I, Coca Menchero S. Clear cell syringoid eccrine carcinoma.  Am J Dermatopathol. 1987;  9 225-231
  • 3 McKee P H, Fletcher C D, Rasbridge S A. The enigmatic eccrine epithelioma (eccrine syringomatous carcinoma).  Am J Dermatopathol. 1990;  12 552-561
  • 4 Alessi E, Caputo R. Syringomatous carcinoma of the scalp presenting as a slowly enlarging patch of alopecia.  Am J Dermatopathol. 1993;  15 503-505
  • 5 Cooper P H, Mills S E, Leonard D D et al.. Sclerosing sweat duct (syringomatous) carcinoma.  Am J Surg Pathol. 1985;  9 422-433
  • 6 Gianotti R, Grimalt R, Alessi E, Caputo R. Spiralled variant of syringomatous carcinoma.  Am J Dermatopathol. 1993;  15 568-571
  • 7 Abenoza P, Ackerman A B. Syringomatous carcinomas. In: Abenoza P, Ackerman AB Neoplasms with Eccrine Differentiation. Ackerman's Histologic Diagnosis of Neoplastic Skin Diseases: A Method by Pattern Analysis Philadelphia, London; Lea & Febiger 1990: 373-412
  • 8 Glatt H J, Proia A D, Tsoy E A et al.. Malignant syringoma of the eyelid.  Ophthalmology. 1984;  91 987-990
  • 9 Hoppenreijs V P, Reuser T T, Mooy C M, Keizer R JW, Mourits M P. Syringomatous carcinoma of the eyelid and orbit: a clinical and histopathological challenge.  Br J Ophthalmol. 1997;  81 668-672
  • 10 Khalil M, Brownstein S, Codere F, Nicolle D. Eccrine sweat gland carcinoma in the eyelid with orbital involvement.  Arch Ophthalmol. 1980;  98 2210-2214
  • 11 Rosen Y, Kim B, Yermakov V A. Eccrine sweat gland tumor of clear cell origin involving the eyelids.  Cancer. 1975;  36 1034-1041
  • 12 Urso C, Bondi R, Paglierani M, Salvadori A, Anichini C. Carcinomas of sweat glands.  Arch Pathol Lab Med. 2001;  125 498-505
  • 13 Ishikawa D, Azumi A, Ohbayashi C, Yamamoto M. A case of syringomatous carcinoma derived from the lid [in Japanese].  Jpn J Ophthalmol. 1998;  102 281-286
  • 14 Hesse R J, Scharfenberg J C, Ratz J L, Griener E. Eyelid microcystic adnexal carcinoma.  Arch Ophthalmol. 1995;  113 494-496
  • 15 Hunts J H, Patel B CK, Langer P D, Anderson R J, Gerwels J W. Microcystic adnexal carcinoma of the eyebrow and eyelid.  Arch Ophthalmol. 1995;  113 1332-1333
  • 16 Leboit P E, Sexton M. Microcystic adnexal carcinoma of the skin.  J Am Acad Dermatol. 1993;  29 609-618
  • 17 Mayer M H, Winton G B, Smith A C, Lupton G P, Parry E L, Shagets F W. Microcystic adnexal carcinoma (sclerosing sweat duct carcinoma).  Plast Reconstr Surg. 1989;  84 970-975
  • 18 Yuh W TC, Egelken J D, Whitaker D C, Dolan K D. Bone marrow invasion of microcystic adnexal carcinoma.  Ann Otol Rhinol Laryngol. 1991;  100 601-603
  • 19 El-Domeiri A A, Richard F RCS, Brasfield R D, Huvos A G, Strong E W. Sweat gland carcinoma: a clinico-pathologic study of 83 patients.  Ann Surg. 1971;  173 270-274
  • 20 Kramer T R, Grossniklaus H E, McLean I W et al.. Histiocytoid variant of eccrine sweat gland carcinoma of the eyelid and orbit.  Ophthalmology. 2002;  109 553-559

Ken-ichi NibuM.D. Ph.D. 

Department of Otolaryngology-Head and Neck Surgery

Graduate School of Medicine, Kobe University

Kusunoki-cho 7-5-1, Chuo-ku, Kobe 650-0017, Japan

Email: nibu@med.kobe-u.ac.jp

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