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Cutaneous CD30 positive large T cell lymphoma of the upper lip: a rare presentation
( 1997I 35, 193- 195
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CutaneousCD30 positive large T cell lymphoma of the upper lip: a rare presentation A. Chandu, D. A. Mitchell, A. M. Corrigan Department of Oral and Maxillofucial
Surgery, Leeds Dental Institute, Clarendon Way, Leeds
SUMMAR Y. Primary cutaneous CD30 positive large T cell lymphomas (PCLCL) are very rare in the head and neck region. We report a case which presented on the right upper lip in a 48-year-old male. This was an isolated cutaneous lesion and in these circumstances this otherwise aggressive lymphoma has an excellent prognosis.
INTRODUCTION Lymphomas are defined as tumours arising from malignant transformation of extramedullary white cells. They are classified as Hodgkin or non-Hodgkin and can be derived from a variety of leucocytes or their precursors. T cell lymphomas are considered to be unusual in Europe and America and make up about 10% of all non-Hodgkin 1ymphomas.l Primary cutaneous CD30 positive large T cell lymphomas (PCLCL) comprise 14% of all primary cutaneous T cell lymphomas’ (CTCL). When identified as isolated skin lesions, these tumours tend to have a favourable prognosis whilst in the presence of nodal disease, the prognosis is poor. Treatment includes local radiotherapy or local excision for small. isolated cutaneous lesions while systemic chemotherapy is reserved for disseminated disease. Little has been written about PCLCL occurring in the head and neck region, especially on the face. In view of this, we present a case report of a 48-yearold male with PCLCL affecting the upper lip.
Fig. 1 -Close up photograph of the lesion incisional biopsy was submitted as a fresh specimen. This showed a diffuse cellular infiltrate through muscle fibres of orbicularis oris. The infiltrate cells resembled lymphocytes, although both the nuclei and the cytoplasm showed pleomorphism. Immunohistochemistry showed the tumour to be CD30, CD5 and CD2 positive. It was concluded that the tumour was a large cell lymphoma with a highly unusual T cell phenotype (Figs 2 and 3). He was taken to the combined Head and Neck Oncology centre to discuss
Case report A 48-year-old nousmoker, presented in early 1995 with a crusted swelling of the right upper lip, adjacent to the commisure (Fig. 1). This swelling had been present for 4-6 weeks after he had ‘nicked his lip’ during shaving and had not responded to antibiotic therapy prescribed by his medical practitioner. On presentation, he was apyrexial and systemically well. His past and present medical history were unremarkable. The lesion was a non-fluctuant, firm, non-tender swelling in a well defined area 2 cm by 2 cm. There was no lymphadenopathy. A provisional diagnosis of an abscesscaused by a staphylococcal infection was made. Treatment consisted of drainage and high dose flucloxacillin and metronidazole. An outpatient appointment was given for 2 days later. With no improvement at the follow-up appointment, an incisional biopsy was carried out under local anaesthesia. The histopathological report described a poorly differentiated malignant neoplasm, suggestive of carcinoma but its origin was unknown and the diagnosis was not conclusive. Because of the uncertainty of the diagnosis, a second
Fig. 2 -
Photomicrograph of the lesion. Bundles are present in a heterogeneous tumour infiltrate magnification x 250).
193
of muscle fibres (H. & E. Original
194
British
Journal
of Oral
and Maxillofacial
Surgery
3 - Photomicrograph using the CD30 antigen stain. Stained tumour cells are to the left while some pale, unstained epithelium is to the right (Original magnification x 250).
Fig.
Fig. 4
-Early
post-treatment photograph.
further management. Total body scan, marrow trephines and blood proteins indicated the lip lesion to be solitary and definitive treatment consisted of localised radiotherapy, thus preserving the comrnisure. He remains well after 9 months (Fig. 4). DISCUSSION
Both the site and age of onset were highly unusual in this instance. The normal age range is between the sixth and seventh decades. There is a male predominance with a male to female ratio of approximately 2 : 1.3 PCLCL can occur anywhere on the skin, although there has been little presented in the literature about these tumours occuring in the head and neck region. PCLCL are defined according to the presence of CD30 positive cells. The malignant lymphoma should be of the T cell phenotype with a predominance (>75%) of large clusters of CD30 positive blast cells in the initial biopsy.2 Histologically, the CD30 positive cells are either anaplastic or pleomorphic with a wide variation in nuclear appearance and an abundance of cytoplasm. These cells are usually in clusters with small inflammatory cells composed of lymphocytes, histocyctes and eosinophils found at the periph-
ery. Reed-Sternberg like cells are often present as are numerous mitotic figures. The use of antigenic markers aids the definitive diagnosis of PCLCL from other CTCL. These lymphomas are strongly CD30 positive and have an aberrant CD4 phenotype. Other activation antigens including CD25 T9, and HLA DR are often positive for HELA 452. CD2, CD3 and CD5 antigens may be lost while CD15 and epithelial membrane antigen (EMA) are not expressed.2*3 PCLCL clinically present as solitary or multiple circumscribed lesions confined to skin. They may present as a large ulcerating tumour. To confirm the diagnosis, there should be no evidence of other primary cutaneous T cell lymphomas present i.e. lymphomatoid papulosis or mycosis fungoides. The lesion in this patient coincided with these criteria. PCLCL have been reported to regress spontaneously but there are no known parameters to indicate which do so. The development of disease in regional lymph nodes has been known to occur in a small number of patients.2 Within the spectrum of CTCL, the classification of such tumours has changed. With the updated Kiel classification,4 PCLCL, both anaplastic and pleomorphic varieties, are classified as high grade lymphomas according to cell size and this type of grading system has also been used by others5 Recently, a new proposal for the classification of lymphoid neoplasms by the International Lymphoma Study Group has been published6 and PCLCL have been placed into the anaplastic large cell lymphoma category. It is interesting to note that the authors acknowledge that they were unable to reproducibly subclassify CTCL. Originally it was thought that subtype (i.e. pleomorphic versus anaplastic) and position (solitary vs diffuse spread) determined prognosis. Beljaards et ~1.~described no significant difference in prognosis between the two histological subtypes and that the spectrum of various primary cutaneous CD30 positive lymphomas is closely related.2,7 Both subtypes now have a favourable prognosis.2q7,s Willenze et d2 have now reclassified this group of T cell lymphomas as low grade malignant CTCL. The PCLCL group have a 4 year survival rate in the region of 90°,6.2,7,8CTCL associated with nodes or disseminated disease carries a poor prognosis. The change in classification may clarify why such lymphomas are rare in the head and neck. PCLCL was first described in the early eighties. A similar lymphoma, Mycosis fungoides has been described on the face, but these reports are few. A case report has recently been published describing a cutaneous T cell lymphoma presenting as a facial swelling9 but this was not a solitary lesion and an outdated classification of the types of T cell lymphoma was used. It is now established that PCLCL is a distinct entity from Mycosis fungoides or Sezary syndrome.2 It is interesting to note that some authorslo have hypothesised a common viral aetiology for Mycosis fungoides, Lymphomatoid papulosis and PCLCL involving the Human T-cell Lymphotrophic retrovirus (HTLV-1). There has also been evidence that
Cutaneous CD30 positive large T cell lymphoma of the upper lip
the Epstein-Barr virus may also be invo1ved.l’ A definitive viral aetiology for these tumours is, however, yet to be established. The treatment of PCLCL depends on the staging of the lesion. Localised radiotherapy or local excision are the preferred methods of treatment for solitary lesions while chemotherapy is reserved for disseminated disease. This patient’s lesion was solitary with no extracutaneous involvement. This case presented the dilemma of having to choose between radiotherapy and complex surgery each having morbidity. In this instance consensus opinion dictated that radiotherapy was the treatment of choice and his prognosis is considered favourable. Acknowledgements The authors would like to thank Dr Alec High for his histopathological input and the Department of Medical and Dental illustration at Leeds Dental Institute for their help in the production of the figures.
References I. Underwood JCE. General and systemic pathology. 1st ed. Edinburgh: Churchill Livingstone, 1992. 2. Willemze R, Beljaards RC and Meijer CJL. Classification of primary cutaneous T cell lymphomas. Histopathol 1994; 24: 405-415. 3. Beljaards RC, Kaudewitz P, Berti E et al. Primary cutaneous CD30 positive large cell lymphomas: definition of a new type of cutaneous lymphoma with a favourable prognosis. A European multicentre study on 47 cases.Cancer 1993; 71: 2097-2103. 4. Stansfield AG, Diebold J, Kapanci Y et al. Updated Kiel classification for lymphomas. Lancet 1988; 1: 292-293.
195
5. Suchi T, Lennert K, Tu L-Y et ul. Histopathology and immunohistochemistry of lymphomas: a proposal for their classification. J Clin Path01 1987; 40: 995-1015. 6. Chan JKC, Banks PM, Cleary ML et 01. A proposal for classification of lymphoid neoplasms (by the International Lymphoma Study Group). Histopathol 1994; 25: 517-536. 7. Willemze R, Beljaards RC. Spectrum of primary cutaneous CD30 (Ki-1)-positive lymphoproliferative disorders. A proposal for classification and guidelines for management and treatment. J Am Acad Dermatol 1993; 28: 973-980. 8. Banerjee SS, Heald J, Harris L. Twelve cases of Ki-I positive anaplastic large cell lymphoma of the skin. J Clin Path01 1991; 44: 119-125. 9. Thomas DW, Lewis MAO, Cowpe JG. Cutaneous lymphoma presenting as a facial swelling: report of a case and review of the literature. Int J Oral Maxillofac Surg 1994; 23: 356-358. 10. Kadin ME. Common activated helper-T cell origin for lymphomatoid papulosis, mycosis fungoides and some types of Hodgkin’s disease. Lancet 1985; 2: 864-865. 11. Slater D. Epstein-Barr virus: An aetiological factor in cutaneous lymphoproliferative disorders (Editorial). J Path01 1991; 165: l-4.
The Authors A. Chandu BDSc House Surgeon D. A. Mitchell FDSRCPS, FRCS Senior Registrar A. M. Corrigan FDSRCPS, FRCS Consultant Department of Oral and Maxillofacial Leeds Dental Institute Clarendon Way Leeds LS2 9LU
Surgery
Correspondence and requests for offprints to Mr A. Chandu Paper received 26 April 1995 Accepted 19 June 1995
I
-
I
CutaneousCD30 positive large T cell lymphoma of the upper lip: a rare presentation A. Chandu, D. A. Mitchell, A. M. Corrigan Department of Oral and Maxillofucial
Surgery, Leeds Dental Institute, Clarendon Way, Leeds
SUMMAR Y. Primary cutaneous CD30 positive large T cell lymphomas (PCLCL) are very rare in the head and neck region. We report a case which presented on the right upper lip in a 48-year-old male. This was an isolated cutaneous lesion and in these circumstances this otherwise aggressive lymphoma has an excellent prognosis.
INTRODUCTION Lymphomas are defined as tumours arising from malignant transformation of extramedullary white cells. They are classified as Hodgkin or non-Hodgkin and can be derived from a variety of leucocytes or their precursors. T cell lymphomas are considered to be unusual in Europe and America and make up about 10% of all non-Hodgkin 1ymphomas.l Primary cutaneous CD30 positive large T cell lymphomas (PCLCL) comprise 14% of all primary cutaneous T cell lymphomas’ (CTCL). When identified as isolated skin lesions, these tumours tend to have a favourable prognosis whilst in the presence of nodal disease, the prognosis is poor. Treatment includes local radiotherapy or local excision for small. isolated cutaneous lesions while systemic chemotherapy is reserved for disseminated disease. Little has been written about PCLCL occurring in the head and neck region, especially on the face. In view of this, we present a case report of a 48-yearold male with PCLCL affecting the upper lip.
Fig. 1 -Close up photograph of the lesion incisional biopsy was submitted as a fresh specimen. This showed a diffuse cellular infiltrate through muscle fibres of orbicularis oris. The infiltrate cells resembled lymphocytes, although both the nuclei and the cytoplasm showed pleomorphism. Immunohistochemistry showed the tumour to be CD30, CD5 and CD2 positive. It was concluded that the tumour was a large cell lymphoma with a highly unusual T cell phenotype (Figs 2 and 3). He was taken to the combined Head and Neck Oncology centre to discuss
Case report A 48-year-old nousmoker, presented in early 1995 with a crusted swelling of the right upper lip, adjacent to the commisure (Fig. 1). This swelling had been present for 4-6 weeks after he had ‘nicked his lip’ during shaving and had not responded to antibiotic therapy prescribed by his medical practitioner. On presentation, he was apyrexial and systemically well. His past and present medical history were unremarkable. The lesion was a non-fluctuant, firm, non-tender swelling in a well defined area 2 cm by 2 cm. There was no lymphadenopathy. A provisional diagnosis of an abscesscaused by a staphylococcal infection was made. Treatment consisted of drainage and high dose flucloxacillin and metronidazole. An outpatient appointment was given for 2 days later. With no improvement at the follow-up appointment, an incisional biopsy was carried out under local anaesthesia. The histopathological report described a poorly differentiated malignant neoplasm, suggestive of carcinoma but its origin was unknown and the diagnosis was not conclusive. Because of the uncertainty of the diagnosis, a second
Fig. 2 -
Photomicrograph of the lesion. Bundles are present in a heterogeneous tumour infiltrate magnification x 250).
193
of muscle fibres (H. & E. Original
194
British
Journal
of Oral
and Maxillofacial
Surgery
3 - Photomicrograph using the CD30 antigen stain. Stained tumour cells are to the left while some pale, unstained epithelium is to the right (Original magnification x 250).
Fig.
Fig. 4
-Early
post-treatment photograph.
further management. Total body scan, marrow trephines and blood proteins indicated the lip lesion to be solitary and definitive treatment consisted of localised radiotherapy, thus preserving the comrnisure. He remains well after 9 months (Fig. 4). DISCUSSION
Both the site and age of onset were highly unusual in this instance. The normal age range is between the sixth and seventh decades. There is a male predominance with a male to female ratio of approximately 2 : 1.3 PCLCL can occur anywhere on the skin, although there has been little presented in the literature about these tumours occuring in the head and neck region. PCLCL are defined according to the presence of CD30 positive cells. The malignant lymphoma should be of the T cell phenotype with a predominance (>75%) of large clusters of CD30 positive blast cells in the initial biopsy.2 Histologically, the CD30 positive cells are either anaplastic or pleomorphic with a wide variation in nuclear appearance and an abundance of cytoplasm. These cells are usually in clusters with small inflammatory cells composed of lymphocytes, histocyctes and eosinophils found at the periph-
ery. Reed-Sternberg like cells are often present as are numerous mitotic figures. The use of antigenic markers aids the definitive diagnosis of PCLCL from other CTCL. These lymphomas are strongly CD30 positive and have an aberrant CD4 phenotype. Other activation antigens including CD25 T9, and HLA DR are often positive for HELA 452. CD2, CD3 and CD5 antigens may be lost while CD15 and epithelial membrane antigen (EMA) are not expressed.2*3 PCLCL clinically present as solitary or multiple circumscribed lesions confined to skin. They may present as a large ulcerating tumour. To confirm the diagnosis, there should be no evidence of other primary cutaneous T cell lymphomas present i.e. lymphomatoid papulosis or mycosis fungoides. The lesion in this patient coincided with these criteria. PCLCL have been reported to regress spontaneously but there are no known parameters to indicate which do so. The development of disease in regional lymph nodes has been known to occur in a small number of patients.2 Within the spectrum of CTCL, the classification of such tumours has changed. With the updated Kiel classification,4 PCLCL, both anaplastic and pleomorphic varieties, are classified as high grade lymphomas according to cell size and this type of grading system has also been used by others5 Recently, a new proposal for the classification of lymphoid neoplasms by the International Lymphoma Study Group has been published6 and PCLCL have been placed into the anaplastic large cell lymphoma category. It is interesting to note that the authors acknowledge that they were unable to reproducibly subclassify CTCL. Originally it was thought that subtype (i.e. pleomorphic versus anaplastic) and position (solitary vs diffuse spread) determined prognosis. Beljaards et ~1.~described no significant difference in prognosis between the two histological subtypes and that the spectrum of various primary cutaneous CD30 positive lymphomas is closely related.2,7 Both subtypes now have a favourable prognosis.2q7,s Willenze et d2 have now reclassified this group of T cell lymphomas as low grade malignant CTCL. The PCLCL group have a 4 year survival rate in the region of 90°,6.2,7,8CTCL associated with nodes or disseminated disease carries a poor prognosis. The change in classification may clarify why such lymphomas are rare in the head and neck. PCLCL was first described in the early eighties. A similar lymphoma, Mycosis fungoides has been described on the face, but these reports are few. A case report has recently been published describing a cutaneous T cell lymphoma presenting as a facial swelling9 but this was not a solitary lesion and an outdated classification of the types of T cell lymphoma was used. It is now established that PCLCL is a distinct entity from Mycosis fungoides or Sezary syndrome.2 It is interesting to note that some authorslo have hypothesised a common viral aetiology for Mycosis fungoides, Lymphomatoid papulosis and PCLCL involving the Human T-cell Lymphotrophic retrovirus (HTLV-1). There has also been evidence that
Cutaneous CD30 positive large T cell lymphoma of the upper lip
the Epstein-Barr virus may also be invo1ved.l’ A definitive viral aetiology for these tumours is, however, yet to be established. The treatment of PCLCL depends on the staging of the lesion. Localised radiotherapy or local excision are the preferred methods of treatment for solitary lesions while chemotherapy is reserved for disseminated disease. This patient’s lesion was solitary with no extracutaneous involvement. This case presented the dilemma of having to choose between radiotherapy and complex surgery each having morbidity. In this instance consensus opinion dictated that radiotherapy was the treatment of choice and his prognosis is considered favourable. Acknowledgements The authors would like to thank Dr Alec High for his histopathological input and the Department of Medical and Dental illustration at Leeds Dental Institute for their help in the production of the figures.
References I. Underwood JCE. General and systemic pathology. 1st ed. Edinburgh: Churchill Livingstone, 1992. 2. Willemze R, Beljaards RC and Meijer CJL. Classification of primary cutaneous T cell lymphomas. Histopathol 1994; 24: 405-415. 3. Beljaards RC, Kaudewitz P, Berti E et al. Primary cutaneous CD30 positive large cell lymphomas: definition of a new type of cutaneous lymphoma with a favourable prognosis. A European multicentre study on 47 cases.Cancer 1993; 71: 2097-2103. 4. Stansfield AG, Diebold J, Kapanci Y et al. Updated Kiel classification for lymphomas. Lancet 1988; 1: 292-293.
195
5. Suchi T, Lennert K, Tu L-Y et ul. Histopathology and immunohistochemistry of lymphomas: a proposal for their classification. J Clin Path01 1987; 40: 995-1015. 6. Chan JKC, Banks PM, Cleary ML et 01. A proposal for classification of lymphoid neoplasms (by the International Lymphoma Study Group). Histopathol 1994; 25: 517-536. 7. Willemze R, Beljaards RC. Spectrum of primary cutaneous CD30 (Ki-1)-positive lymphoproliferative disorders. A proposal for classification and guidelines for management and treatment. J Am Acad Dermatol 1993; 28: 973-980. 8. Banerjee SS, Heald J, Harris L. Twelve cases of Ki-I positive anaplastic large cell lymphoma of the skin. J Clin Path01 1991; 44: 119-125. 9. Thomas DW, Lewis MAO, Cowpe JG. Cutaneous lymphoma presenting as a facial swelling: report of a case and review of the literature. Int J Oral Maxillofac Surg 1994; 23: 356-358. 10. Kadin ME. Common activated helper-T cell origin for lymphomatoid papulosis, mycosis fungoides and some types of Hodgkin’s disease. Lancet 1985; 2: 864-865. 11. Slater D. Epstein-Barr virus: An aetiological factor in cutaneous lymphoproliferative disorders (Editorial). J Path01 1991; 165: l-4.
The Authors A. Chandu BDSc House Surgeon D. A. Mitchell FDSRCPS, FRCS Senior Registrar A. M. Corrigan FDSRCPS, FRCS Consultant Department of Oral and Maxillofacial Leeds Dental Institute Clarendon Way Leeds LS2 9LU
Surgery
Correspondence and requests for offprints to Mr A. Chandu Paper received 26 April 1995 Accepted 19 June 1995