Pustular mycosis fungoides has a poor outcome: a multicentric clinico-pathological and molecular case series study
Christophe Bontoux
(1, 2, 3, 4)
,
Marine Badrignans
(5)
,
Sivem Afach
(6)
,
Emilie Sbidian
(6)
,
Diana-Laure Mboumba
(7, 8)
,
Saskia Ingen-Housz-Oro
(6, 7)
,
Alexis Claudel
(9, 10, 11)
,
Marie-Hélène Aubriot-Lorton
(12)
,
Arnaud Chong-Si-Tsaon
(13)
,
Gilles Le Masson
(14)
,
Christophe Attencourt
(15, 16)
,
Romain Dubois
(17)
,
Fanny Beltzung
(18)
,
Wafa Koubaa
(19)
,
Helmut Beltraminelli
(20)
,
Nathalie Cardot-Leccia
(21)
,
Brigitte Balme
(22)
,
Anh Tuan Nguyen
(23)
,
Kelly Bagny
(13)
,
Delphine Legoupil
(24)
,
Ibtissam Moustaghfir
(25)
,
Juliette Denamps
(26)
,
Laurent Mortier
(17)
,
Houda Hammami-Ghorbel
(19)
,
Sergey Skrek
(27)
,
Mostefa Rafaa
(28)
,
Anne-Claire Fougerousse
(23)
,
Thibaut Deschamps
(29)
,
Stéphane Dalle
(30)
,
Michel D’incan
(31)
,
Guillaume Chaby
(26)
,
Marie Beylot-Barry
(32)
,
Sophie Dalac
(33)
,
Nicolas Ortonne
(5, 8)
1
IUCT Oncopole - UMR 1037 -
Institut Universitaire du Cancer de Toulouse - Oncopole
2 CRCT - Centre de Recherches en Cancérologie de Toulouse
3 Laboratoire de Pathologie Clinique et Expérimentale. Hôpital Pasteur [Nice]
4 UNICANCER/CAL - Centre de Lutte contre le Cancer Antoine Lacassagne [Nice]
5 Département de pathologie [Mondor]
6 EpiDermE - Epidemiology in Dermatology and Evaluation in Therapeutics
7 Service de dermatologie [Mondor]
8 IMRB - Institut Mondor de Recherche Biomédicale
9 CHU Henri Mondor [Créteil]
10 UPEC UP12 - Université Paris-Est Créteil Val-de-Marne - Paris 12
11 U955 Inserm - UPEC - IMRB - NFL/"Neurofibromatosis and Lymphoma Oncogenesis" [Créteil]
12 Service de Pathologie [CHU de Dijon]
13 Centre hospitalier Félix-Guyon [Saint-Denis, La Réunion]
14 CHU - BREST - Biologie pathologie - CHRU Brest, Pôle de Biologie Pathologie
15 Anatomie et Cytologie Pathologiques [CHU Amiens]
16 CHIMERE - CHirurgie, IMagerie et REgénération tissulaire de l’extrémité céphalique - Caractérisation morphologique et fonctionnelle - UR UPJV 7516
17 Hôpital Claude Huriez [Lille]
18 Centre médico chirurgical Magellan - Hôpital Haut-Lévêque - CHU de Bordeaux
19 Hôpital Habib Thameur [Tunis]
20 Inselspital Bern
21 CHU - Hôpital Pasteur [Nice]
22 CHLS - Centre Hospitalier Lyon Sud [CHU - HCL]
23 HIA Begin - Hôpital d'Instruction des Armées Begin [Saint-Mandé, France]
24 CHRU Brest - Centre Hospitalier Régional Universitaire de Brest
25 Service de Dermatologie [CHU Nantes]
26 Service de dermatologie [CHU d'Amiens-Picardie]
27 North Western State Medical University Named After I.I Mechnikoff
28 Centre Hospitalier Sud Francilien
29 Centre hospitalier de Valence
30 UNICANCER/CRCL - Centre de Recherche en Cancérologie de Lyon
31 Service Dermatologie et Oncologie Cutanée [CHU Clermont-Ferrand]
32 Service de dermatologie Hôpital Saint-André Bordeaux
33 Service de Dermatologie (CHU de Dijon)
2 CRCT - Centre de Recherches en Cancérologie de Toulouse
3 Laboratoire de Pathologie Clinique et Expérimentale. Hôpital Pasteur [Nice]
4 UNICANCER/CAL - Centre de Lutte contre le Cancer Antoine Lacassagne [Nice]
5 Département de pathologie [Mondor]
6 EpiDermE - Epidemiology in Dermatology and Evaluation in Therapeutics
7 Service de dermatologie [Mondor]
8 IMRB - Institut Mondor de Recherche Biomédicale
9 CHU Henri Mondor [Créteil]
10 UPEC UP12 - Université Paris-Est Créteil Val-de-Marne - Paris 12
11 U955 Inserm - UPEC - IMRB - NFL/"Neurofibromatosis and Lymphoma Oncogenesis" [Créteil]
12 Service de Pathologie [CHU de Dijon]
13 Centre hospitalier Félix-Guyon [Saint-Denis, La Réunion]
14 CHU - BREST - Biologie pathologie - CHRU Brest, Pôle de Biologie Pathologie
15 Anatomie et Cytologie Pathologiques [CHU Amiens]
16 CHIMERE - CHirurgie, IMagerie et REgénération tissulaire de l’extrémité céphalique - Caractérisation morphologique et fonctionnelle - UR UPJV 7516
17 Hôpital Claude Huriez [Lille]
18 Centre médico chirurgical Magellan - Hôpital Haut-Lévêque - CHU de Bordeaux
19 Hôpital Habib Thameur [Tunis]
20 Inselspital Bern
21 CHU - Hôpital Pasteur [Nice]
22 CHLS - Centre Hospitalier Lyon Sud [CHU - HCL]
23 HIA Begin - Hôpital d'Instruction des Armées Begin [Saint-Mandé, France]
24 CHRU Brest - Centre Hospitalier Régional Universitaire de Brest
25 Service de Dermatologie [CHU Nantes]
26 Service de dermatologie [CHU d'Amiens-Picardie]
27 North Western State Medical University Named After I.I Mechnikoff
28 Centre Hospitalier Sud Francilien
29 Centre hospitalier de Valence
30 UNICANCER/CRCL - Centre de Recherche en Cancérologie de Lyon
31 Service Dermatologie et Oncologie Cutanée [CHU Clermont-Ferrand]
32 Service de dermatologie Hôpital Saint-André Bordeaux
33 Service de Dermatologie (CHU de Dijon)
Emilie Sbidian
- Fonction : Auteur
- PersonId : 774565
- ORCID : 0000-0002-1267-5270
Saskia Ingen-Housz-Oro
- Fonction : Auteur
- PersonId : 814296
- ORCID : 0000-0002-5383-7096
Stéphane Dalle
- Fonction : Auteur
- PersonId : 937229
- IdHAL : stephanedalle
- ORCID : 0000-0002-3423-4548
- IdRef : 075987279
Guillaume Chaby
- Fonction : Auteur
- PersonId : 1143945
- ORCID : 0000-0003-0172-2892
- IdRef : 074246445
Marie Beylot-Barry
- Fonction : Auteur
- PersonId : 762790
- ORCID : 0000-0001-6150-1229
- IdRef : 05956427X
Résumé
Background Mycosis fungoides (MF) has usually an indolent course. However, some patients develop a more aggressive disease and few prognostic parameters have been identified. Isolated cases of pustular MF (pMF) suggest an unfavourable prognosis. Objectives We aim to describe the clinico-pathological characteristics and prognostic value of pMF. Methods We retrospectively collected data of all cases of MF with histological pustules diagnosed from 2009 to 2020. The outcomes and clinico-pathological characteristics of pMF at diagnosis (pMFD) were compared to those of a cohort of non-pustular MF (NpMF). Results 33 pMF (including 22 pMFD) and 86 NpMF cases were included. The median age at diagnosis of pMF was 61 years [IQR=50-75]. The median follow-up of pMFD was 32 months [IQR=14-49]. Clinically, 33% of pMF had pustules. Large-cell transformation (LCT) occurred in 17 cases. pMFD were at a significantly more advanced-stage and more showed LCT at diagnosis than NpMF (50% vs 7%, p<0.001 and 23% vs 0%, p<0.001, respectively). In multivariate Cox analysis, the presence of histological pustule at diagnostic was associated with shorter OS in all patients (HR=13.90, CI95%[2.43-79]; p=0.003), and in early-stage patients (HR=11.09, CI95%[1.56-78.82]; p=0.02). In multivariate Fine and Gray model analysis, pMFD was associated with a higher cumulative incidence of LCT (SHR=13.90, CI95% [2.43-79]; p=0.003) in all patients. Median OS after the occurrence of histological pustules during follow-up of all pMF patients was 37 months, with a five-year OS of 25% (CI95% [0.06-0.5]). Conclusion pMF often follows an aggressive course, with a high risk of LCT and shorter survival, even for early-stage patients. Histological pustules at diagnostic of MF might represent an independent poor prognostic factor, to be confirmed by further studies. Because pustules are not always clinically identified, histological pustules should be mentioned in pathology reports of MF and prompt discussion of a closer follow-up.