Incidental Diagnosis of Lymphangioleiomyomatosis (LAM) in Gynecological Surgery – A Case Series
Bibliographic record
Abstract
Background : Lymphangioleiomyomatosis (LAM) is a rare, slow progressing, low-grade neoplasm that primarily effects premenopausal women. The disease is of systemic nature and well known for its pulmonary involvement with cystic destruction, but extra-pulmonary disease may occur and present as renal angiomyolipoma or lymphangioleiomyomtous lesions in different organs. LAM-associated lesions may present mutations in the tuberous sclerosis complex gens and may develop sporadically or in the context of tuberous sclerosis as hereditary disease. Incident LAM may represent the sentinel finding of the disease. Methods : A data base research was performed for LAM in gynecological surgical specimens. H&E-stained slides were re-examined for exact localization of the LAM within the specimen. Immunohistochemical stains were reevaluated for expression patterns. Clinical data were retrieved for the presence of tuberous sclerosis. Results : The age ranged from 32 to 78 years (mean=54 yrs, median=55 yrs), with 8/13 were≤ 55 years. One woman had a history of LAM with lung involvement and another woman had clinical diagnosis of tuberous sclerosis. Overall, no history of renal angiomyolipoma or PEComa noted. 9/13 women underwent surgery for gynecological malignancy, 2/13 for endometriosis and complex atypical endometrium hyperplasia and 2/13 for pelvic discomfort and pelvic lymphadenopathy. On histological examination 10/13 women incidentally presented LAM involvement in 1 to 9 lymph nods (median=1,5) with a lesion size of 0,5 to 12 mm (median=2,3 mm), mainly located subcapsular or in the nodal parenchyma, one half with extranodal spread in the fatty tissue. 3/13 women showed extranodal involvement of the retroperitoneum, myometrium, and the hilum of the ovary. The neoplasm stained in most cases positive for HMB45 (patchy 9/13, focal 1/13), for desmin (4/7) and smooth muscle actin (3/3), with negativity for borad spectrum cytokeratins (6/6) and S100 (3/3). LAM was associated with tuberous sclerosis in 2/13 patients. Conclusion : LAM is a rare systemic disease, which mainly involves the lungs. Nevertheless, abdominal nodal or extranodal manifestations may occur and therefore should be considered as a differential diagnosis in the spectrum of abdominal spindle cell neoplasm. It is important to report the incidental finding of even small foci of LAMs as a clue for women with yet unrecognized tuberous sclerosis complex. Publication History Article published online: 11 June 2024 © 2024. Thieme. All rights reserved. Georg Thieme Verlag KG Rüdigerstraße 14, 70469 Stuttgart, Germany
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How this classification was reachedexpand
Full frame machine prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.
Distilled classifier scores by category (both heads)
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.000 | 0.004 |
| Meta-epidemiology (narrow) | 0.001 | 0.001 |
| Meta-epidemiology (broad) | 0.001 | 0.001 |
| Bibliometrics | 0.003 | 0.002 |
| Science and technology studies | 0.003 | 0.002 |
| Scholarly communication | 0.001 | 0.002 |
| Open science | 0.001 | 0.002 |
| Research integrity | 0.004 | 0.002 |
| Insufficient payload (model declined to judge) | 0.003 | 0.001 |
Machine scores (provisional)
The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.
Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from itClassification
machine, unvalidatedMachine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.
How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".