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Primary CD30+ lymphoproliferative disorder of the vulva

Primary CD30+ lymphoproliferative disorder of the vulva. Narciso Mariani, Paola Barbieri, Paola Re, Sara Orecchia, Pier-Giacomo Betta S.C. Anatomia Patologica, Azienda Ospedaliera Nazionale di Alessandria. Introduzione.

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Primary CD30+ lymphoproliferative disorder of the vulva

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  1. Primary CD30+ lymphoproliferative disorder of the vulva Narciso Mariani, Paola Barbieri, Paola Re, Sara Orecchia, Pier-Giacomo Betta S.C. Anatomia Patologica, Azienda Ospedaliera Nazionale di Alessandria

  2. Introduzione Primary non-Hodgkin lymphomas (NHL) of the female genital tract are rare and those in the vulva are extremely uncommon (117 cases reported in a survey of the literature up to 2005), mostly featuring the B immunophenotype (particularly, diffuse large cell and follicular NHL).

  3. A 17-year-old female was submitted in July 2008 to an excisional biopsy of a solitary ulcer of the labius major of the vulva of  0,5 cm. in its greatest diameter, of a few months duration and non-responsive to local anti-inflammatory therapy. Histologically the lesion showed a skin ulcer with the epidermis replaced by a thick layer of necrotic material overlying a recently-formed granulation tissue extensively involving the dermis.

  4. E.E. x200 E.E. x20 E.E. x400

  5. In the necrotic material, there were multiple foci of a surviving cell popolation arranged in a perivascular pattern and consisting of medium- to large-sized lymphoid cells featuring the following immunophenotype: CD3+, CD45R0 +, CD30+, CD1a-, CD20-, CD43-, CD56, Alk- and EMA-. CD3 x400 CD30 x200

  6. In situ RNA hybridisation for EBV (EBER ISH) was negative and PCR analysis revealed no clonal rearrangement of either TCR or heavy chain Ig. The final histopathological diagnosis was CD30 + T-cell lymphoproliferative disorders with unpredictable biological behaviour. EBER ISH x100 A close follow-up was recommended, which in the following 6 months revealed no further lesions, either local or at distance.

  7. Bibliografia • Kempf W. CD30+ lymphoproliferative disorders: histopathology, differential diagnosis, new variants, and simulators. J Cutan Pathol 2006; 33 (Suppl. 1): 58-70. • Kosari F et al. Lymphomas of the female genital tract. Am J Surg Pathol 2005; 29 (11): 1512- 1520. • Martorell M et al. Transformation of vulvar pseudolymphoma (lymphoma like-lesion) into a marginal zone B-cell lymphoma of labium majus. J Obstet Gynaecol Res 2008; 34 (4 part II): 699-705.

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