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Intra-abdominal desmoplastic small cell tumour in a patient with Peutz-Jeghers syndrome

Abstract

A surgical case of intra-abdominal desmoplastic small cell tumour with divergent differentiation (IDS-CT) in a patient with Peutz-Jeghers syndrome (PJS) is reported. The patient was a 23-year-old woman who underwent surgery for a tumour in the left paraovarian region. Widespread peritoneal metastases were noted. Histopathological examination revealed solid cell nests composed of medium-sized cells demarcated by desmoplastic stroma. Gland-like spaces were found within many cell nests. Tumour cells were positive for cytokeratin and smooth muscle actin, but negative for desmin. Both ovaries contained minute foci of a sex cord tumour with annular tubules. This is the first documentation of the association of IDSCT with PJS. Since patients with PJS have an increased risk of cancer in various organs, the occurrence of IDSCT in the present patient might not be fortuitous.

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References

  1. 1.

    Burdick D, Prior JT (1982) Peutz-Jeghers syndrome. A clinicopathologic study of a large family with a 27-year follow-up. Cancer 50:2139–2146

  2. 2.

    Chadha S, Kwast TH van der (1989) Immunohistochemistry of ovarian granulosa cell tumours. The value of tissue specific proteins and tumour markers. Virchows Arch [A] 414:439–445

  3. 3.

    Cruikshank SH, Drie DM van (1982) Supernumerary ovaries. Update and review. Obstet Gynecol 60:126–129

  4. 4.

    Dickersin GR, Kline IW, Scully RE (1982) Small cell carcinoma of the ovary with hypercalcemia. A report of eleven cases. Cancer 49:188–197

  5. 5.

    Dozois RR, Kempers RD, Dahlin DC, Bartholomew LG (1970) Ovarian tumors associated with the Peutz-Jeghers syndrome. Ann Surg 172:233–238

  6. 6.

    Gerald WL, Rosai J (1989) Desmoplastic small cell tumor with divergent differentiation. Pediatr Pathol 9:177–183

  7. 7.

    Gerald WL, Miller HK, Battifora H, Miettinen M, Silva EG, Rosai J (1991) Intra-abdominal desmoplastic small round-cell tumor. Report of 19 cases of a distinctive type of high-grade polyphenotypic malignancy affecting young individuals. Am J Surg Pathol 15:499–513

  8. 8.

    Gonzalez-Crussi F, Crawford SE, Sun CCJ (1990) Intraabdominal desmoplastic small-cell tumors with divergent differentiation. Observations on three cases of childhood. Am J Surg Pathol 14:633–642

  9. 9.

    Hsu SD, Zaharopoulos P, May JT, Costanzi JJ (1979) Peutz-Jeghers syndrome with intestinal carcinoma. Report of the association in one family. Cancer 44:1527–1532

  10. 10.

    Layfield LJ, Lenarsky C (1991) Desmoplastic small cell tumors of the peritoneum coexpressing mesenchymal and epithelial markers. Am J Clin Pathol 96:536–543

  11. 11.

    McCaughey WTE, Kannerstein M, Churg J (1985) Tumors and pseudotumors of the serous membranes. Atlas of tumor pathology, 2nd series, fascicle 20. Armed Forces Institute of Pathology, Washington D.C.

  12. 12.

    McGowan L, Young RH, Scully RE (1980) Peutz-Jeghers syndrome with “adenoma malignum” of the cervix. A report of two cases. Gynecol Oncol 10:125–133

  13. 13.

    Ordóñez NG, Zirkin R, Bloom RE (1989) Malignant small-cell epithelial tumor of the peritoneum coexpressing mesenchymal-type intermediate filaments. Am J Surg Pathol 13:413–421

  14. 14.

    Ordóñez NG, El-Naggar AK, Ro JY, Silva EG, MacKay B (1993) Intra-abdominal desmoplastic small cell tumor. A light microscopic, immunocytochemical, ultrastructural, and flow cytometric study. Hum Pathol 24:850–865

  15. 15.

    Scully RE (1970) Sex cord tumor with annular tubules. A distinctive ovarian tumor of the Peutz-Jeghers syndrome. Cancer 25:1107–1121

  16. 16.

    Trau H, Schewach-Millet M, Fisher BK, Tsur H (1982) Peutz-Jeghers syndrome and bilateral breast carcinoma. Cancer 50:788–792

  17. 17.

    Variend S, Gerrard M, Norris PD, Goepel JR (1991) Intra-abdominal neuroectodermal tumour of childhood with divergent differentiation. Histopathology 18:45–51

  18. 18.

    Wills EJ (1993) Peritoneal desmoplastic small round cell tumours with divergent differentiation. A review. Ultrastruct Pathol 17:295–306

  19. 19.

    Young RH, Welch WR, Dickersin R, Scully RE (1982) Ovarian sex cord tumor with annular tubules. Review of 74 cases including 27 with Peutz-Jeghers syndrome and four with adenoma malignum of the cervix. Cancer 50:1384–1402

  20. 20.

    Young RH, Dickersin GR, Scully RE (1983) A distinctive ovarian sex cord-stromal tumor causing sexual precocity in the Peutz-Jeghers syndrome. Am J Surg Pathol 7:233–243

  21. 21.

    Young RH, Eichhorn JH, Dickersin GR, Scully RE (1992) Ovarian involvement by the intra-abdominal desmoplastic small round cell tumor with divergent differentiation. A report of three cases. Hum Pathol 23:454–464

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Correspondence to M. Shintaku.

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Shintaku, M., Baba, Y. & Fujiwara, T. Intra-abdominal desmoplastic small cell tumour in a patient with Peutz-Jeghers syndrome. Vichows Archiv A Pathol Anat 425, 211–215 (1994). https://doi.org/10.1007/BF00230359

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Key words

  • Desmoplastic small cell tumour
  • Peutz-Jeghers syndrome