Diffuse Sclerosing Variant of Papillary Thyroid Carcinoma Is Associated With Aggressive Histopathological Features and a Poor Outcome: Results of a Large Multicentric Study.
Diffuse sclerosing variant (DSV) is a rare and aggressive subtype of papillary thyroid carcinoma (PTC).



The objective of the study was to investigate the clinicopathological features and prognosis of DSV patients and compare these findings with all other PTCs and high-risk PTCs.



The data of patients who underwent surgery for DSV and PTC between 2003 and 2014 in seven surgical departments specialized in endocrine surgery were reviewed.



Fifty-six DSV patients were included (mean age 32.6 ± 12.5 y; 46 [82%] female) and were compared with 2945 non-DSV PTCs and 48 high-risk PTCs.



Forty-six DSV patients (82%) were pT3, 43 (77%) had an extrathyroidal extension, and 54 (96%) had lymph node metastasis, including 48 patients with involvement in the lateral compartment (86%). During the follow-up period of 4.3 ±2.3 years, 19 patients (34%) had a recurrence, including 18 patients with an ipsilateral lateral compartment recurrence. The only prognostic factor for recurrence in the multivariate analysis was extranodal extension (odds ratio 3.4 [1.1; 10.8]). The 7-year recurrence-free survival (RFS) was 63%. The RFS was significantly worse in patients with DSV than in other PTC patients (hazard risk 8.5 [5.2; 13.9], P < .0001) and were similar to the RFS of high-risk PTCs (hazard risk 1.1 [0.6; 2.2], P = .5).



DSV patients share the same recurrence rate as high-risk PTC patients. Despite aggressive surgical approaches, the recurrence rate within the first 5 years requires a careful ongoing surveillance, similar to the follow-up of high-risk PTC patients.
Departments of General and Endocrine Surgery (N.C., X.G., C.T., F.M.), Hospital Pitié Salpêtrière, Pierre et Marie Curie University, 75013 Paris, France; University Hospital of Lille (F.P.), 59037 Lille, France; University Hospital Lyon Sud (J.-C.L.), 69310 Lyon, France; University Hospitals of Geneva (F.Tr.), 1211 Geneva, Switzerland; University Hospital Hôtel-Dieu (E.M.), 44093 Nantes, France; University Hospital of Dijon (P.G.), 21079 Dijon, France; University Hospital of Nancy (L.B.), 54511 Nancy, France; Departments of Pathology (F.Ti.), Endocrinology (L.L.), and Biostatistics, and Unité Mixte de Recherche en Santé 1136, Institut Pierre Louis d'Epidémiologie et de Santé Publique (T.d.M.), Hospital Pitié Salpêtrière, Pierre et Marie Curie University, 75013 Paris, France.
Nathalie
N
Chereau
Departments of General and Endocrine Surgery (N.C., X.G., C.T., F.M.), Hospital Pitié Salpêtrière, Pierre et Marie Curie University, 75013 Paris, France; University Hospital of Lille (F.P.), 59037 Lille, France; University Hospital Lyon Sud (J.-C.L.), 69310 Lyon, France; University Hospitals of Geneva (F.Tr.), 1211 Geneva, Switzerland; University Hospital Hôtel-Dieu (E.M.), 44093 Nantes, France; University Hospital of Dijon (P.G.), 21079 Dijon, France; University Hospital of Nancy (L.B.), 54511 Nancy, France; Departments of Pathology (F.Ti.), Endocrinology (L.L.), and Biostatistics, and Unité Mixte de Recherche en Santé 1136, Institut Pierre Louis d'Epidémiologie et de Santé Publique (T.d.M.), Hospital Pitié Salpêtrière, Pierre et Marie Curie University, 75013 Paris, France.
Xavier
X
Giudicelli
Departments of General and Endocrine Surgery (N.C., X.G., C.T., F.M.), Hospital Pitié Salpêtrière, Pierre et Marie Curie University, 75013 Paris, France; University Hospital of Lille (F.P.), 59037 Lille, France; University Hospital Lyon Sud (J.-C.L.), 69310 Lyon, France; University Hospitals of Geneva (F.Tr.), 1211 Geneva, Switzerland; University Hospital Hôtel-Dieu (E.M.), 44093 Nantes, France; University Hospital of Dijon (P.G.), 21079 Dijon, France; University Hospital of Nancy (L.B.), 54511 Nancy, France; Departments of Pathology (F.Ti.), Endocrinology (L.L.), and Biostatistics, and Unité Mixte de Recherche en Santé 1136, Institut Pierre Louis d'Epidémiologie et de Santé Publique (T.d.M.), Hospital Pitié Salpêtrière, Pierre et Marie Curie University, 75013 Paris, France.
Francois
F
Pattou
Departments of General and Endocrine Surgery (N.C., X.G., C.T., F.M.), Hospital Pitié Salpêtrière, Pierre et Marie Curie University, 75013 Paris, France; University Hospital of Lille (F.P.), 59037 Lille, France; University Hospital Lyon Sud (J.-C.L.), 69310 Lyon, France; University Hospitals of Geneva (F.Tr.), 1211 Geneva, Switzerland; University Hospital Hôtel-Dieu (E.M.), 44093 Nantes, France; University Hospital of Dijon (P.G.), 21079 Dijon, France; University Hospital of Nancy (L.B.), 54511 Nancy, France; Departments of Pathology (F.Ti.), Endocrinology (L.L.), and Biostatistics, and Unité Mixte de Recherche en Santé 1136, Institut Pierre Louis d'Epidémiologie et de Santé Publique (T.d.M.), Hospital Pitié Salpêtrière, Pierre et Marie Curie University, 75013 Paris, France.
Jean-Christophe
JC
Lifante
Departments of General and Endocrine Surgery (N.C., X.G., C.T., F.M.), Hospital Pitié Salpêtrière, Pierre et Marie Curie University, 75013 Paris, France; University Hospital of Lille (F.P.), 59037 Lille, France; University Hospital Lyon Sud (J.-C.L.), 69310 Lyon, France; University Hospitals of Geneva (F.Tr.), 1211 Geneva, Switzerland; University Hospital Hôtel-Dieu (E.M.), 44093 Nantes, France; University Hospital of Dijon (P.G.), 21079 Dijon, France; University Hospital of Nancy (L.B.), 54511 Nancy, France; Departments of Pathology (F.Ti.), Endocrinology (L.L.), and Biostatistics, and Unité Mixte de Recherche en Santé 1136, Institut Pierre Louis d'Epidémiologie et de Santé Publique (T.d.M.), Hospital Pitié Salpêtrière, Pierre et Marie Curie University, 75013 Paris, France.
Frederic
F
Triponez
Departments of General and Endocrine Surgery (N.C., X.G., C.T., F.M.), Hospital Pitié Salpêtrière, Pierre et Marie Curie University, 75013 Paris, France; University Hospital of Lille (F.P.), 59037 Lille, France; University Hospital Lyon Sud (J.-C.L.), 69310 Lyon, France; University Hospitals of Geneva (F.Tr.), 1211 Geneva, Switzerland; University Hospital Hôtel-Dieu (E.M.), 44093 Nantes, France; University Hospital of Dijon (P.G.), 21079 Dijon, France; University Hospital of Nancy (L.B.), 54511 Nancy, France; Departments of Pathology (F.Ti.), Endocrinology (L.L.), and Biostatistics, and Unité Mixte de Recherche en Santé 1136, Institut Pierre Louis d'Epidémiologie et de Santé Publique (T.d.M.), Hospital Pitié Salpêtrière, Pierre et Marie Curie University, 75013 Paris, France.
Eric
E
Mirallié
Departments of General and Endocrine Surgery (N.C., X.G., C.T., F.M.), Hospital Pitié Salpêtrière, Pierre et Marie Curie University, 75013 Paris, France; University Hospital of Lille (F.P.), 59037 Lille, France; University Hospital Lyon Sud (J.-C.L.), 69310 Lyon, France; University Hospitals of Geneva (F.Tr.), 1211 Geneva, Switzerland; University Hospital Hôtel-Dieu (E.M.), 44093 Nantes, France; University Hospital of Dijon (P.G.), 21079 Dijon, France; University Hospital of Nancy (L.B.), 54511 Nancy, France; Departments of Pathology (F.Ti.), Endocrinology (L.L.), and Biostatistics, and Unité Mixte de Recherche en Santé 1136, Institut Pierre Louis d'Epidémiologie et de Santé Publique (T.d.M.), Hospital Pitié Salpêtrière, Pierre et Marie Curie University, 75013 Paris, France.
Pierre
P
Goudet
Departments of General and Endocrine Surgery (N.C., X.G., C.T., F.M.), Hospital Pitié Salpêtrière, Pierre et Marie Curie University, 75013 Paris, France; University Hospital of Lille (F.P.), 59037 Lille, France; University Hospital Lyon Sud (J.-C.L.), 69310 Lyon, France; University Hospitals of Geneva (F.Tr.), 1211 Geneva, Switzerland; University Hospital Hôtel-Dieu (E.M.), 44093 Nantes, France; University Hospital of Dijon (P.G.), 21079 Dijon, France; University Hospital of Nancy (L.B.), 54511 Nancy, France; Departments of Pathology (F.Ti.), Endocrinology (L.L.), and Biostatistics, and Unité Mixte de Recherche en Santé 1136, Institut Pierre Louis d'Epidémiologie et de Santé Publique (T.d.M.), Hospital Pitié Salpêtrière, Pierre et Marie Curie University, 75013 Paris, France.
Laurent
L
Brunaud
Departments of General and Endocrine Surgery (N.C., X.G., C.T., F.M.), Hospital Pitié Salpêtrière, Pierre et Marie Curie University, 75013 Paris, France; University Hospital of Lille (F.P.), 59037 Lille, France; University Hospital Lyon Sud (J.-C.L.), 69310 Lyon, France; University Hospitals of Geneva (F.Tr.), 1211 Geneva, Switzerland; University Hospital Hôtel-Dieu (E.M.), 44093 Nantes, France; University Hospital of Dijon (P.G.), 21079 Dijon, France; University Hospital of Nancy (L.B.), 54511 Nancy, France; Departments of Pathology (F.Ti.), Endocrinology (L.L.), and Biostatistics, and Unité Mixte de Recherche en Santé 1136, Institut Pierre Louis d'Epidémiologie et de Santé Publique (T.d.M.), Hospital Pitié Salpêtrière, Pierre et Marie Curie University, 75013 Paris, France.
Christophe
C
Trésallet
Departments of General and Endocrine Surgery (N.C., X.G., C.T., F.M.), Hospital Pitié Salpêtrière, Pierre et Marie Curie University, 75013 Paris, France; University Hospital of Lille (F.P.), 59037 Lille, France; University Hospital Lyon Sud (J.-C.L.), 69310 Lyon, France; University Hospitals of Geneva (F.Tr.), 1211 Geneva, Switzerland; University Hospital Hôtel-Dieu (E.M.), 44093 Nantes, France; University Hospital of Dijon (P.G.), 21079 Dijon, France; University Hospital of Nancy (L.B.), 54511 Nancy, France; Departments of Pathology (F.Ti.), Endocrinology (L.L.), and Biostatistics, and Unité Mixte de Recherche en Santé 1136, Institut Pierre Louis d'Epidémiologie et de Santé Publique (T.d.M.), Hospital Pitié Salpêtrière, Pierre et Marie Curie University, 75013 Paris, France.
Frédérique
F
Tissier
Departments of General and Endocrine Surgery (N.C., X.G., C.T., F.M.), Hospital Pitié Salpêtrière, Pierre et Marie Curie University, 75013 Paris, France; University Hospital of Lille (F.P.), 59037 Lille, France; University Hospital Lyon Sud (J.-C.L.), 69310 Lyon, France; University Hospitals of Geneva (F.Tr.), 1211 Geneva, Switzerland; University Hospital Hôtel-Dieu (E.M.), 44093 Nantes, France; University Hospital of Dijon (P.G.), 21079 Dijon, France; University Hospital of Nancy (L.B.), 54511 Nancy, France; Departments of Pathology (F.Ti.), Endocrinology (L.L.), and Biostatistics, and Unité Mixte de Recherche en Santé 1136, Institut Pierre Louis d'Epidémiologie et de Santé Publique (T.d.M.), Hospital Pitié Salpêtrière, Pierre et Marie Curie University, 75013 Paris, France.
Laurence
L
Leenhardt
Departments of General and Endocrine Surgery (N.C., X.G., C.T., F.M.), Hospital Pitié Salpêtrière, Pierre et Marie Curie University, 75013 Paris, France; University Hospital of Lille (F.P.), 59037 Lille, France; University Hospital Lyon Sud (J.-C.L.), 69310 Lyon, France; University Hospitals of Geneva (F.Tr.), 1211 Geneva, Switzerland; University Hospital Hôtel-Dieu (E.M.), 44093 Nantes, France; University Hospital of Dijon (P.G.), 21079 Dijon, France; University Hospital of Nancy (L.B.), 54511 Nancy, France; Departments of Pathology (F.Ti.), Endocrinology (L.L.), and Biostatistics, and Unité Mixte de Recherche en Santé 1136, Institut Pierre Louis d'Epidémiologie et de Santé Publique (T.d.M.), Hospital Pitié Salpêtrière, Pierre et Marie Curie University, 75013 Paris, France.
Sophie Tezenas
ST
du Montcel
Departments of General and Endocrine Surgery (N.C., X.G., C.T., F.M.), Hospital Pitié Salpêtrière, Pierre et Marie Curie University, 75013 Paris, France; University Hospital of Lille (F.P.), 59037 Lille, France; University Hospital Lyon Sud (J.-C.L.), 69310 Lyon, France; University Hospitals of Geneva (F.Tr.), 1211 Geneva, Switzerland; University Hospital Hôtel-Dieu (E.M.), 44093 Nantes, France; University Hospital of Dijon (P.G.), 21079 Dijon, France; University Hospital of Nancy (L.B.), 54511 Nancy, France; Departments of Pathology (F.Ti.), Endocrinology (L.L.), and Biostatistics, and Unité Mixte de Recherche en Santé 1136, Institut Pierre Louis d'Epidémiologie et de Santé Publique (T.d.M.), Hospital Pitié Salpêtrière, Pierre et Marie Curie University, 75013 Paris, France.
Fabrice
F
Menegaux
D000328:Adult; D002277:Carcinoma / Q000473:pathology* / Q000601:surgery; D002291:Carcinoma, Papillary; D018572:Disease-Free Survival; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D009361:Neoplasm Invasiveness; D009364:Neoplasm Recurrence, Local / Q000473:pathology*; D017063:Outcome Assessment, Health Care; D012306:Risk; D000077273:Thyroid Cancer, Papillary; D013964:Thyroid Neoplasms / Q000473:pathology* / Q000601:surgery