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Anatomic pathology selected abstracts

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Geisler DL, Nestler RJ, Mosley BL, et al. Accuracy of definitive rapid onsite evaluation cytopathology diagnoses: Assessment of potentially critical diagnoses as a quality assurance measure. J Am Soc Cytopathol. 2022;11:133–141.

Correspondence: Dr. N. Paul Ohori at ohorinp@upmc.edu

Pathology and prognosis of colonic adenocarcinomas with intermediate primary tumor stage

Primary tumor stage is an important prognostic indicator in colonic adenocarcinomas. However, tumors lacking muscle fibers beyond the advancing tumor edge and that show no extension beyond the apparent outer border of the muscularis propria, termed pT2int, have not been previously studied, according to the authors. The authors conducted a study to address the clinicopathologic characteristics and prognosis of pT2int tumors. They recharacterized 168 colon carcinomas and compared pT2int tumors with pT2 and pT3 tumors. In side-by-side analyses, 21 pT2int tumors diverged from 29 pT2 tumors only in terms of being larger (P = 0.03). However, when compared with 118 pT3 tumors, the pT2int tumors were less likely to show high grade (P = 0.03); lymphovascular (P < 0.001) and extramural venous invasion (P = 0.04); discontinuous tumor deposits (P = 0.02); lymph node involvement (P = 0.001); and advanced stage (P = 0.001). Combining pT2int with pT2 cases was a better independent predictor of negative lymph nodes in multivariate analysis (P = 0.04; odds ratio [OR], 3.96; confidence interval [CI], 1.09–14.42) and absent distant metastasis in univariate analysis (P = 0.04) compared with sorting pT2int and pT3 cases. Proportional hazards regression showed that pT2 and pT2int tumors together were associated with better disease-free survival than pT3 tumors (P = 0.04; OR, 3.65; 95 percent CI, 1.05–12.70). Kaplan-Meier analysis demonstrated that when pT2int and pT2 tumors were grouped together, those patients were significantly less likely to show disease progression than patients with pT3 tumors (P = 0.002; log-rank test) and showed a trend toward better disease-specific survival (P = 0.06) during a mean follow-up of 44.9 months. These data support the conclusion that pT2int carcinomas have distinct clinicopathologic characteristics and are associated with patient outcomes more closely aligned with pT2 versus pT3 tumors.

Paulsen JD, Polydorides AD. Pathology and prognosis of colonic adenocarcinomas with intermediate primary tumor stage between pT2 and pT3. Arch Pathol Lab Med. 2022;146(5):591–602.

Correspondence: Dr. Alexandros D. Polydorides at alexandros.polydorides@mountsinai.org

Kaposiform lymphangiomatosis: pathologic aspects in 43 patients

Kaposiform lymphangiomatosis is an uncommon generalized lymphatic anomaly with distinctive clinical, radiologic, histopathologic, and molecular findings. The authors documented the pathology in 43 patients evaluated by the Boston Children’s Hospital Vascular Anomalies Center from 1999 to 2020. The most frequent presentations were respiratory difficulty, hemostatic abnormalities, and a soft tissue mass. Imaging commonly revealed involvement of some combination of the mediastinal, pulmonary, pleural, and pericardial compartments and most often included the spleen and skeleton. Histopathology was characterized by dilated, redundant, and abnormally configured lymphatic channels typically accompanied by dispersed clusters of variably canalized and often hemosiderotic spindled lymphatic endothelial cells that were immunopositive for D2-40, PROX1, and CD31. An activating lesional NRAS variant was documented in nine of 10 patients. The clinical course was typically aggressive and marked by hemorrhage, thrombocytopenia, diminished fibrinogen levels, and a mortality rate of 21 percent. The authors concluded that it is critical to diagnose kaposiform lymphangiomatosis because it often has a progressive course. A diagnosis is best achieved via an interdisciplinary team. Biopsies or molecular studies should be undertaken if deemed necessary. Molecular alterations in kaposiform lymphangiomatosis validate the use of RAS-MAPK pathway inhibitors, which have shown promise in isolated cases.

Perez-Atayde AR, Debelenko L, Al-Ibraheemi A, et al. Kaposiform lymphangiomatosis: pathologic aspects in 43 patients. Am J Surg Pathol.2022;46(7):963–976.

Correspondence: Dr. Harry P. W. Kozakewich at harry.kozakewich@childrens.harvard.edu

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