Co‐existence of multiple myeloma and perivascular endothelial cell tumor
Bibliographic record
Abstract
A 56-year-old woman presented with a left upper thigh mass and associated leg weakness. On work-up, she was found to be anemic (hemoglobin 51 g/L) with hypercalcemia (3.49 mmol/L), serum free light chain spike, Bence Jones protein, and a compression fracture of L2. A peripheral blood smear showed rouleaux and rare circulating plasma cells. A bone marrow aspirate was hemodilute but contained many plasma cells (Panel A). A bone marrow biopsy (B) showed 80% plasma cells which were stained positive for CD138 (C), kappa (D), CD56, and negative for lambda (E). This was consistent with a diagnosis of multiple myeloma (MM). Molecular cytogenetic studies were negative for high-risk myeloma genetic abnormalities. Clinically, the leg mass was initially thought to represent an extramedullary manifestation of MM. However, a biopsy of the leg mass showed a high-grade malignant pleomorphic tumor comprising polygonal cells with large irregular nuclei, fine chromatin, prominent nucleoli, and abundant eosinophilic cytoplasm (F). The tumor cells were positive for TFE3 (G) and MIFT (H) with focal positivity for SMA (I), S100, and Melan-A/HMB45 cocktail, negative for CD138. A diagnosis of malignant perivascular endothelial cell tumor (PEComa) was made (Figure 1). Although patients with MM and coexisting with a different primary neoplasm (either hematological or solid organ neoplasm) have previously been described,1 to our knowledge, this is the first report of concurrent MM and PEComa. PEComas are a distinctive mesenchymal neoplasm arising from perivascular epithelioid cells that usually demonstrate combined myomelanocytic immunophenotype. Most PEComas are sporadic, however, and can be associated with tuberous sclerosis complex harboring deletions of TSC2 gene with the activation of mTOR pathway.2 Co-existence of MM with solid tumors seems to appear sporadically, whereby genetic predisposition, tumor-induced immunodeficiency, deregulated release of cytokines, and chronic inflammation may contribute to such associations.1 It is unclear whether these two neoplasms were linked as no further molecular testing was performed in this case. This case also highlights an important clinical consideration that unrelated solid tumors can co-exist in the setting of MM that might mimic extramedullary disease. The authors have no competing interests.
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How this classification was reachedexpand
Full frame distilled prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. Learned from the 10,348 direct Codex labels and 10,348 direct Gemma labels. Candidate is the union of thresholded teacher heads; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels or direct frontier model labels.
Codex and Gemma teacher scores by category
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.000 | 0.001 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.000 |
| Bibliometrics | 0.000 | 0.000 |
| Science and technology studies | 0.000 | 0.000 |
| Scholarly communication | 0.000 | 0.000 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.000 | 0.000 |
| Insufficient payload (model declined to judge) | 0.000 | 0.000 |
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 teacher head, 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".