Nasal peripheral T-cell lymphoma: A 20-year review of cases treated in Scotland

doi:10.1016/S0936-6555(05)80974-4

Clinical Oncology

Volume 4, Issue 2, 1992, Pages 96-100

https://doi.org/10.1016/S0936-6555(05)80974-4 Get rights and content

The nasal peripheral (post-thymic) T-cell lymphoma is an important cause of the midline granuloma syndrome (MGS), in which ulceration and destruction of the tissues of the nose and paranasal sinuses occurs. We reviewed the histology of 9 cases of the MGS treated with radiotherapy, and, using immunocytochemistry, showed 8 cases to be peripheral T-cell lymphomas (PTCL) and 1 a B-cell lymphoma.

All patients received radiotherapy and 2 died shortly after treatment from unrelated causes. Two patients with T-cell lymphoma and the solitary case of B-cell lymphoma achieved long-term disease-free survival. The 4 remaining cases of T-cell lymphoma relapsed locally at a median interval of 3.5 months despite megavoltage irradiation of 45–50 Gy (in 3 cases) and inclusion of uninvolved paranasal sinuses and the nasopharynx in the field (in 2 cases). All patients with local relapse achieved, and remain in, remission after treatment with alkylating agents and prednisolone.

The disappointing response of some cases of nasal T-cell lymphoma to radiotherapy has been reported by others, and this may be due partly to the heterogeneity of nasal lymphomas. We are unable to provide clear guidelines for treatment but suggest that a role exists for initial treatment with oral alkylating agents and steroids in newly diagnosed cases.

References (29)

ChottA. et al.
Peripheral T-cell lymphoma presenting primarily as lethal midline granuloma
Hum Pathol
(1988)
HoF.C.S. et al.
Polymorphic reticulosis and conventional lymphomas of the nose and upper aerodigestive tract: A clinicopathologic study of 70 cases, and immunophenotypic studies of 16 cases
Hum Pathol
(1990)
ItamiJ. et al.
Non-Hodgkin's lymphoma confined to the nasal cavity: its relationship to the polymorphic reticulosis and results of radiation therapy
Int J Radiat Oncol Biol Phy
(1991)
O'ConnorN.T.J. et al.
Rearrangement of the T-cell receptor beta-chain gene in the diagnosis of lymphoproliferative disorders
Lancet
(1985)
SimrellC.R. et al.
Lymphokine induced phagocytosis in angiocentric immunoproliferative lesions (AIL) and malignant lymphoma arising in AIL
Blood
(1985)
HarabuchiY. et al.
Epstein-Barr virus in nasal T-cell lymphomas in patients with the lethal midline granuloma
Lancet
(1990)
VenningM.C. et al.
Antibodies to neutrophil cytoplasmic antigen in systemic vasculitis [letters]
Lancet
(1987)
SmalleyS. et al.
Polymorphic reticulosis limited to the upper aerodigestive tract: natural history and radiotherapeutic considerations
Int J Radiat Oncol Biol Phys
(1988)
CostaJ. et al.
The midline granuloma syndrome
Pathol Annu
(1986)
YamanakaN. et al.
Midfacial T-cell lymphoma: characterisation by monoclonal antibodies
Ann Otol Rhinol Laryngol
(1985)

ChanJ.K.C. et al.

Most nasal/nasopharyngeal lymphomas are peripheral T-cell neoplasms

Am J Surg Pathol

(1987)

SuchiT. et al.

Histopathology and immunohistochemistry of peripheral T-cell lymphomas: a proposal for their classification

J Clin Pathol

(1987)

AozasaK. et al.

Nationwide study of lethal midline granuloma in Japan: frequencies of Wegener's granulomatosis, polymorphic reticulosis, malignant lymphoma and other related conditions

Int J Cancer

(1989)

SternbergerL.A. et al.

The unlabelled antibody enzyme method of immunocytochemistry

Preparation and properties of soluble antigen-antibody complex (horseradish peroxidase) and its use in identification of spirochaetes

J Histochem Cytochem

(1970)

Cited by (12)

Diagnostic Pathology: Head & Neck
2017, Diagnostic Pathology: Head and Neck
Early or Up-Front Radiotherapy Improved Survival of Localized Extranodal NK/T-Cell Lymphoma, Nasal-Type in the Upper Aerodigestive Tract
2008, International Journal of Radiation Oncology Biology Physics
Citation Excerpt :
In the current study, consisting of 63.4% with Stage I disease, 5-year OS and DFS were 52.3% and 41.0% for all patients, respectively. These were comparable to the previous reports, which quoted a 5-year OS ranging from 34% to 86%, and a DFS or PFS ranging from 32% to 57% (10–23, 27, 28). Patients receiving RT in our study had much better OS and DFS compared with patients receiving CT alone.
To investigate the role of early or up-front radiotherapy (RT), the optimal RT dose required to achieve appropriate treatment outcome and prognostic factors for patients with localized extranodal NK/T-cell lymphoma, nasal-type, in the upper aerodigestive tract.
Eighty-two patients were reviewed. Eight patients were treated with chemotherapy (CT) alone, 9 patients received RT alone, and 65 patients were given combined modality treatment of CT and RT (CMT). Of those 74 patients receiving RT, 31 patients were given up-front RT, whereas CT was the initial therapy for 43 patients and 41 of those 43 patients received early RT.
Five-year overall survival (OS) and disease-free survival (DFS) were 52.3% and 39.2%, respectively. RT was the only independent prognostic factor for both OS and DFS at both the univariate and multivariate level. The 5-year OS and DFS were better in patients receiving ≥54 Gy of RT as compared with that of <54 Gy (5-year OS 75.5% vs. 46.1%, p = 0.019; 5-year DFS 60.3% vs. 33.4%, p = 0.004). Up-front RT presented better survival in Stage I patients when compared with that of initial CT followed by early RT (5-year OS 90.0% vs. 48.9%, p = 0.012; 5-year DFS 78.7% vs. 39.9%, p = 0.021).
Early or up-front RT had an essential role in improved OS and DFS in patients with localized extranodal NK/T-cell lymphoma, nasal-type, in the upper aerodigestive tract. The recommended tumor dose was at least 54 Gy. Up-front RT may yield more benefits on survival in patients with Stage I disease.
Experience of radiotherapy in lethal midline granuloma with special emphasis on centrofacial T-cell lymphoma: A retrospective analysis covering a 34-year period
1996, Radiotherapy and Oncology
Lethal midline granuloma (LMG) is characterized by progressive ulceration and destruction of the midfacial tissue. It occurs more frequently in Oriental than in Western populations. Because of the progress in clinical pathology and immunohistochemistry, most cases have been proven to be malignant lymphomas, especially of T-cell lineage. We describe 92 cases of lethal midline granuloma or centrofacial malignant lymphoma in the period 1959–1993. All received complete courses of radiotherapy. Twenty of them also received combination chemotherapy. Thirty-six cases had specimens available for immunohistochemical study; 25 (69%) of these had a T-cell phenotype, and 6 (17%) were of B-cell lineage. The dose to the nasal region was in the range of 3000–7500 cGy in 11–58 days, and to the neck 3000–6400 cGy in 11–48 days. The overall survival rate for the LMGs was 59.5% at 5 years and 56.2% at 10 years (Kaplan-Meier). Combined chemotherapy seemed not to improve the overall survival in this study (p = 0.63), but the patient number was too small to make a firm conclusion. Based on the results of this study, we recommend a dose of 4500–5000 cGy to the midfacial region, since a higher dosage did not improve the treatment results (p = 0.88). Irradiation has a definite role in good locoregional control of this disease. The recent clarification of the disease nature and the recognition of the background clinicopathological features should provide valuable information for future patient management and prospective studies.
International Consensus Statement on Allergy and Rhinology: Sinonasal Tumors
2024, International Forum of Allergy and Rhinology
Intensity-modulated radiotherapy versus three-dimensional conformal radiotherapy for stage I-II natural killer/T-cell lymphoma nasal type: Dosimetric and clinical results
2013, Radiation Oncology
Lethal midline granuloma-nasal natural killer/T-cell lymphoma
2006, American Journal of Clinical Oncology: Cancer Clinical Trials

View all citing articles on Scopus

View full text

Original articleNasal peripheral T-cell lymphoma: A 20-year review of cases treated in Scotland

Hum Pathol

Hum Pathol

Int J Radiat Oncol Biol Phy

Lancet

Blood

Lancet

Lancet

Int J Radiat Oncol Biol Phys

The midline granuloma syndrome

Pathol Annu

Midfacial T-cell lymphoma: characterisation by monoclonal antibodies

Ann Otol Rhinol Laryngol

Most nasal/nasopharyngeal lymphomas are peripheral T-cell neoplasms

Am J Surg Pathol

Histopathology and immunohistochemistry of peripheral T-cell lymphomas: a proposal for their classification

J Clin Pathol

Nationwide study of lethal midline granuloma in Japan: frequencies of Wegener's granulomatosis, polymorphic reticulosis, malignant lymphoma and other related conditions

Int J Cancer

The unlabelled antibody enzyme method of immunocytochemistry

Preparation and properties of soluble antigen-antibody complex (horseradish peroxidase) and its use in identification of spirochaetes

J Histochem Cytochem

Original article
Nasal peripheral T-cell lymphoma: A 20-year review of cases treated in Scotland