Ligneous Cervicitis in a Woman With Plasminogen Deficiency Associated With an Atypical Form of Microglandular Hyperplasia

Abstract

A 19-yr-old woman with previously diagnosed clear cell adenocarcinoma was referred to the Charité for further treatment. Biopsies were taken from the cervix, the endometrium, pseudomembranes in the peritoneum, and sentinel lymph nodes. The morphologic picture of pseudomembranes and inflammation together with the provided information about plasminogen deficiency of the patients led to the hypothesis of ligneous cervicitis. The previously taken biopsies of the adenocarcinoma were reevaluated and showed a clear cell lesion. Further immunohistochemical examination with antibodies against p16, Ki67, CEA, and p53 could not prove its malignant character. As a result we diagnosed an atypical form of microglandular hyperplasia in a patient with ligneous cervicitis. Ligneous cervicitis is a rare inflammatory condition that might affect all mucus membranes in patients with plasminogen deficiency. This case shows the importance of correlating pathologic and clinical findings in the diagnosis of ligneous cervicitis because of the rarity of the disease and the heterogeneity at presentation.

Thrombin and plasmin generation in patients with plasminogen or plasminogen activator inhibitor type 1 deficiency

“…Of the 10 F I G U R E 1 The Nijmegen Hemostasis Assay. (1) lag time, the time at which thrombin formation is initiated; (2) thrombin peak time, that is the time when thrombin production reaches maximal velocity; (3) thrombin peak height, the maximal velocity of thrombin generation; (4) the area under the curve (AUC, relates to the thrombin potential); 5) plasmin peak height, the maximal velocity of plasmin production and; (6) fibrin lysis time (FLT), the time between the initiation of thrombin generation and the time plasmin generation reaches maximal velocity; (7) plasmin potential, area under the curve that represents the total amount of plasmin generated…”

“…Although ligneous conjunctivitis is a characteristic manifestation of PLGD, involvement of the gingiva, oral cavity, tracheobronchial tree, middle ear, gastrointestinal and urogenital tracts, and central nervous system are described. [2][3][4][5][6][7] There are two sub-types of PLGD: type 1 is a quantitative deficiency and type 2 a qualitative deficiency. Patients with type 1 PLGD clinically present with development of pseudomembranes.…”

“…PLGD results in the development of woody pseudomembranes on mucosal surfaces. Although ligneous conjunctivitis is a characteristic manifestation of PLGD, involvement of the gingiva, oral cavity, tracheobronchial tree, middle ear, gastrointestinal and urogenital tracts, and central nervous system are described . There are two sub‐types of PLGD: type 1 is a quantitative deficiency and type 2 a qualitative deficiency.…”

PAX2 and Cyclin D1 Expression in the Distinction Between Cervical Microglandular Hyperplasia and Endometrial Microglandular-like Carcinoma

“…However, rare examples of MGH show atypical features such as a solid or confluent growth pattern, nuclear enlargement and hyperchromasia, hobnail cells, signet ring-like cells, and/or mitotic activity, and these may cause concern for endocervical malignancy (4)(5)(6). However, rare examples of MGH show atypical features such as a solid or confluent growth pattern, nuclear enlargement and hyperchromasia, hobnail cells, signet ring-like cells, and/or mitotic activity, and these may cause concern for endocervical malignancy (4)(5)(6).…”

Atypical Microglandular Hyperplasia of Endocervix as the Presenting Feature of Plasminogen Deficiency

“…In the context of gynecologic pathology, plasminogen deficiency has been associated not only with ligneous disease but also with an atypical endocervical microglandular proliferation that can mimic malignancy both clinically and histologically (7,8). Herein we present an additional case of plasminogen deficiency presenting with endocervical atypical microglandular hyperplasia (MGH) in association with prominent fibrinous exudate in cervical and endometrial biopsy specimens.…”