Volume 3, Issue 2 , Pages 85-89, March 2008
Precursor B-cell lymphoblastic lymphoma presenting as a malignant ear tumour in a five-month-old child
Article Outline
Summary
Ear manifestation of malignant lymphoma is very rare in childhood. The authors present a two-month-old girl with precursor B-cell lymphoblastic lymphoma/leukaemia appearing as a rapid growing malignant right ear tumour. This is the second reported case of childhood lymphoma occurring as an external ear swelling, similar to that which had previously been presented in a two-year-old boy.
The authors comment on the diagnostic and therapeutic problems connected to the uncommon auricular mass. The characteristics of childhood malignant tumours and their therapy are discussed. Needle aspiration is not diagnostic in the case of non-Hodgkin lymphoma (NHL). A surgical sample was taken and histological and immunohistological examinations were made in order to establish a correct diagnosis. The baby received chemotherapy by Interfant-2006 ALL protocol. Two months later the ear tumour had totally disappeared. Authors call the attention of paediatricians and otolaryngologists to the fact that atypical lesions of the head and neck regions may represent a potential for NHL in children.
The importance of team work among the pediatric ORL specialists and pediatric oncologic centres for improving curability rate is emphasized.
Keywords: Malignant lymphoma, Children, External ear, Leukaemia
Introduction
Lymphoma (Hodgkin's and NHL) is a distinct tumour of lymphoid system cells and usually – but not exclusively – occurs in the lymphatic organs [3]. The head and neck localisation of lymphoma is the second most common after abdominal manifestation [1]. The determination of T or B-cell origin and malignancy is helped by immunohistochemical findings [11], [12].
Benoit et al. reported 37 cases of malignant childhood tumours in the head and neck region during a 33-year period [2]. Most of their cases were NHL and rhabdomyosarcoma. The more frequent tumour localisations they mentioned are the nasopharynx, the paranasal sinuses and the soft tissue of the parotid region. There have also been reports of lymphomas of the middle ear, mastoid process, external and internal auditory canal [4], [5], [7], [8], [9], [10]. Tumour manifestations of the pinna are extremely rare [6]. There is only one similar infant case of a two-year-old boy reported previously according to the literature [1].
Now, we present the case of a two-month-old baby girl with a precursor B-cell lymphoblastic lymphoma, describing its diagnostic and therapeutic problems.
Case report
At first, the baby was brought in by her parents to another ORL department because of a little swelling on her right ear. 1.5 months later the baby was examined at the same place again. There was a growing, firm, non-tender swelling covered with light purple skin on the right cavum conchae progressing to the tragus and antitragus and narrowing the external ear canal entrance. An angiological consultation was done, which didn’t support the diagnosis of haemangioma. The tumour was considered to be an atheroma and the surgical removal of the mass was suggested. Before the extirpation, the ORL specialist asked for a needle aspiration.
It unambiguously established a malignant tumour. The histological result raised the possibility of small cell sarcoma, an embrional rhabdoid tumour or sialoblastoma. They excluded the possibility of malignant lymphoma.
Waiting for the histological result, the tumour infiltrated the tragus and the external ear canal. The ear drum became no longer examinable.
The MRI examination of the head showed a 3
×3×2.7cm soft tissue completely filling the external ear canal and progressing to the parotid region. The tumour couldn’t exactly be differentiated from the parotid gland. The cartilage of pinna was seriously thickened and infiltrated.
The CT scan of the pyramid region didn’t show any bone destruction. At the time of the CT examination, repeated aspiration cytology was done to confirm the histological findings but the bloody sample unfortunately wasn’t suitable for the immunohistochemical method.
A haemato-oncologic consultation was made at the Pediatric Clinic, in Budapest on 18th October, 2006 where the first diagnostic examinations (abdominal and heart ultrasound, ECG, chest X-ray, bone scintigraphy) were done with negative results.
Three months had passed from the first manifestation of swelling. There had been several examinations done during that period, but there had been no exact histological result as yet.
After three months the patient was sent to our clinic in order to establish the correct diagnosis. By that time the whole external ear was extremely swollen and the entrance of the outer ear canal couldn’t be seen at all. The process progressed towards the neighbouring areas – the tumour had become unambiguously inoperable (Fig. 1.).
Figure 1.
(A) The photographs taken of the right ear on the first visit at our department and (B) pre-operatively.
The laboratory findings showed no deviation except for elevated leucocyte level. This was a serious dilemma. We had an otherwise healthy five-month-old infant with a clinically malignant, inoperable ear tumour without an exact histological result. Because of these aspects and the fact that aspiration cytology wasn’t successful enough to get a correct histological determination, we decided to take an open biopsy by high frequency radiosurgery (Ellman Dual Surgitron – CUT/COAG mode) (Fig. 2).
Figure 2.
Intra-operative photographs.
The sample was sent to the Pathology Institute Budapest for a histological examination where an experienced team worked on it. The result we received was precursor B-cell lymphoblastic lymphoma. We have to mention that the possibility of that type of tumour was previously out of the question according to aspiration cytology. The histological diagnosis was confirmed by CD79a, CD10, CD20, Ki67, CD3 and CD7 staining (Fig. 3).
Figure 3.
Photos of the histological examination showing precursor B-cell lymphoma: (A) CD10 immunohistochemistry, (B) haematoxylin-eosin, (C) CD79 immunohistochemistry and (D) Ki67 immunohistochemistry.
After receiving the histological result, diagnostic examinations were continued at the Pediatric Clinic in Budapest. There was an 80% premature blast population in the bone marrow, so the diagnosis had to be altered as ALL. The patient is receiving therapy by Interfant-2006 protocol in the oncological centre now. The protocol consists of prednisolone, dexamethasone, vincristine, cytosar, daunomycin, asparaginase, cyclophosphamide, mercaptopurine and methotrexate treatment. She is in the third phase of treatment already and her reactions are very good. After two months of treatment, the infiltration of the external ear has absolutely disappeared and the leukemic cells previously shown on the 15th and 33rd days could not be found by sternum punction any more (Fig. 4).
Figure 4.
The right ear after the third phase of the treatment.
Discussion
Malignant diseases of the lymphocyte-macrophage system are divided into leukaemia and lymphoma according to the organs (bone marrow, blood, lymph nodes) where they occur.
Leukaemia is the expansion of tumour cells in the bone marrow and blood. The bone marrow is overwhelmed by these cells and the blood usually has an enhanced white blood cell number. Nevertheless, sometimes they can’t be found in the blood at all.
Lymphoma is the expansion of lymphoid system cells as an isolated tumour [3].
In a normal case, functionally mature lymphoid cells communicate between lymphatic organs so it may happen that the lymphomas originally located in tissues, alter into leukemic form.
On the other hand, in cases primarily recognised as leukaemia, tumour cells can accumulate in the lymph nodes, spleen or other organs.
These are really two interconnected diseases. When there are more than 25% blast cells in the sternum, the disease is referred to as leukaemia. This doesn’t alter the therapeutic protocol.
The histology of the involved organs (mainly in cases of lymphoma) has crucial importance along with blood picture and bone marrow punction.
The diagnosis and the classification of lymphoma along with cell origin and malignancy determination are mainly based on histological and immunohistochemical examinations. Histological characterization on the basis of cytological preparation is not yet available.
Aspiration cytology is a technologically less complex, but more accurate and sensitive method to differentiate the causes of head and neck swellings (malignant haematological diseases, metastases and reactive or specific inflammations). In cases of adult parotid tumours this is the only currently used diagnostic procedure for differentiation to avoid risk of facial nerve injury, tumour spreading, and contamination. The sensitivity of ultrasound-guided aspiration cytology is nearly 90% when performed by an experienced team, but it is not informative enough to diagnose lymphoma. Aspiration cytology, although technologically less complicated, doesn’t give enough information about the tumour structure. This information is vital to the classification and prognosis of lymphoma.
Besides a physical check-up, an X-ray, CT, MRI and ultrasound examination is important in order to determine the extent of the disease.
There are 250–300 new malignant tumour cases registered every year in Hungary. 25% of these cases are acute lymphoma. Manifestation of the disease under one year of age is very rare. The chromosomal localisation typically found in infancy is the 4:11 translocation which couldn’t be found in our patient. Secondary generalisation of primary lymphoma like in our case is frequently observed.
It is well-known that malignant tumours of infants and children have different locations, histological structures and course than those of adults. Tumours frequently observed in adults (e.g. carcinomas) are rarely observed in childhood. Metastatic carcinoma is frequently observed among adult head and neck swellings of unknown origin, whereas reactive inflammation, NHL, and sarcoma are more often seen in this localization during infancy. It is an important difference that infant malignant tumours have relatively good curability, and chemotherapy plays an important role in their treatment.
Ear manifestations of lymphoma are extremely rare [13]. In our case, the localisation and progression of the tumour had the same behaviour as a malignant parotid tumour or sarcoma at first. This fact altered the diagnostic procedure because specialists were thinking like they usually did during the verification procedures of these other tumours. Due to the unusual early childhood appearance and atypical tumour localization, we had to reconsider the fact that aspiration cytology would be the only effective diagnostic method. The open biopsy proved to be the way to get a correct diagnosis. This case reveals the borderline problem of aspiration cytology and open biopsy in certain situations.
75% of ALL cases have a five year curability rate which is a very good result. Nevertheless infant ALL cases have a worse prognosis than childhood ALL.
Conclusion
The infant and childhood malignant tumours are very different from that of adults, for example tumour localisation, type of tumours and curability.
Head and neck tumours are frequently under differentiated and can occur as part of system diseases. Aspiration cytology is not appropriate for determining cell origin and malignancy in the case of lymphoma. It is important that atypical lesions of the head and neck regions may represent a potential for NHL in children. Sometimes open biopsy may be required to establish a correct diagnosis.
It is suggested that children be treated in pediatric centres where pediatric oncology and otorhinolaryngology is available, and where the treatment of tumours is planned by specific protocols. Due to these facts, the curability rate has been improving from year to year.
References
- . Precursor B-cell lymphoblastic lymphoma presenting as an isolated external ear swelling in a two-year-old child. Int. J. Pediatr. Otorhinolaryngol. 2005;69(May (5)):695–699
- . Malignant otolaryngological tumors in children. Acta Otorhinolaryngol. Belg. 1984;38(3):288–301
- E. László, Laboratory investigation of malignant transformation of lymphocyte-macrophag system in Pathopsysiology Institute of Semmelweis University, Budapest, personal communication.
- . Primary middle-ear lymphoma in a child. J. Laryngol. Otol. 2003;117(March (3)):205–207
- . Extension of nasopharyngeal lymphoma to the middle and external ear. Ann. Otol. Rhinol. Laryngol. 2003;112(July (7)):644–646
- . Systemic B-cell lymphoma presenting as an isolated lesion on the ear. Clin. Exp. Dermatol. 2001;26(March (2)):166–169
- . Non-Hodgkin lymphoma of the infratemporalis fossa: a case report. Rev. Stomatol. Chir. Maxillofac. 2003;104(December (6)):347–351
- . Lymphoma of the tympanic membrane in acquired immunodeficiency syndrome. Auris Nasus Larynx. 1998;25(January (1)):89–94
- . Primary presentation of malignant lymphoma in middle ear cleft. Ann. Otol. Rhinol. Laryngol. 1980;89(March–April (2 Pt 1)):180–183
- . Petrous bone tumors: primary non-Hodgkin lymphoma of the inner ear canal and cerebellopontile angle. Laryngorhinootologie. 1997;76(October (10)):625–628
- . Primary lymphoma of the internal auditory canal. Case report and review of the literature. Ann. Otol. Rhinol. Laryngol. 1998;107(January (1)):17–21
- . Primary lymphoma of bilateral external auditory canals. Am. J. Otolaryngol. 2002;23(January–February (1)):49–52
- . Lymphoma in the ear. ORL J. Otorhinolaryngol. Relat. Spec. 2000;62(September–October (5)):274–277
PII: S1871-4048(07)00091-3
doi:10.1016/j.pedex.2007.11.003
© 2007 Elsevier Ireland Ltd. All rights reserved.
Volume 3, Issue 2 , Pages 85-89, March 2008
