Mediastinal ganglioneuroma with lymph node metastasis in a boy presenting upper airway infection
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《中华医药杂志》英文版
1 Department of Pediatrics; 2 Department of Surgery and Orthopedics;3 Department of Pathology; 4 Department of Dermatology and Radiotherapy, Botucatu Medical School-UNESP, SP, Brazil; 5 Department of Health Sciences, Federal University of Sao Paulo, UNIFESP, SP, Brazil
Correspondence to Daniel A. Ribeiro, DDS, PhD,Departamento de Patologia,Faculdade de Medicina de Botucatu-UNESP,Distrito de Rubio Jr s/n,Botucatu-SP,18618-000,Brazil
Tel: 55 14 38828255,Fax: 55 14 3815 2348,E-mail: araki@fmb.unesp.br
[Abstract] Ganglioneuroma is a benign neurogenic tumour which represents the final maturation stage of neuroblast tumours. In this case report, a mediastinal ganglioneuroma with lymph node metastasis from a five-year-old boy presenting upper airway infection is reported.
[Key words] ganglioneuroma; mediastinal neoplasms; upper airway infection; child
INTRODUCTION
Neuroblast tumours originate of precursor cells from sympathetic nervous system, consisting of the adrenal medulla and the paravertebral sympathetic ganglions [1~3]. They are composed of ganglion nerve and Schwann cells and are able to evolute from the undifferentiated and malignant neuroblastoma undergoing ganglioneuroblastoma, to mature ganglioneuroma [3], which is the most differentiated tumour in this group [1]. Classification in neuroblastoma, mixed ganglioneuroblastoma, nodular ganglioneuroblastoma, and ganglioneuroma is based on the degree of neuroblast differentiation depicted as cellular turnover index, and Schwannian stroma development. Ganglioneuromas are divided into two distinct subtypes: a) in maturation, which already presents disperse accumulation of differentiating neuroblasts, and b) mature, where the neuroblastomatose component is absent.
Ganglioneuroma is the rarest of the neuroblast tumours [4~6], where it is mainly found in the thorax (43%), particularly in the retroperitoneal space, the adrenal medulla, and the soft oropharyngeal tissues [1,4,6]. It affects the over threes, teenagers, and young adults (mean 7 years) with slight predominance in females [4,6,7]. The treatment is surgery only [8].
The goal of this current case report is to present a mediastinal ganglioneuroma with lymph node metastasis in a boy presenting upper airway infection.
CASE REPORT
A 5-year-old boy was admitted to Botucatu Medical School, Clinical Hospital (FMB-UNESP), with a clinical picture of upper airway infection. After submitting to thorax X-ray, a large asymptomatic mass with regular outline was seen occupying the inferior 1/3 of the right hemithorax, in the posterior mediastinum (Figure 1). Also, it was noted a rounded somewhat elevated area, soft to percussion and with reduced stethoscope vesicle murmur, in the lower medial right posterior thoracic region. Abdominal ultrasound showed no abdominal masses and free retroperitonium. Thoracic tomography revealed a posterior mediastinal mass with evidence of infiltration into the costal arch adjacent to the vertebrae (Figure 2). Fine needle puncture aspiration was inconclusive. Thus, an excisional biopsy was indicated by means of right posterior lateral thoracotomy. The diaphragm and median mediastinium were free. However, there was no cleavage plane between the tumour and the intercostal spaces and vertebrae. The rib which seemed to be invaded was only deformed. The cincumjacent lymph nodes were compromised (diagnosed as metastases). They were completely resected with the tumour.
Figure 1 X-ray of thorax revealing large mass with irregular forms in posterior area of mediastinum
Figure 2 Computer tomography of thorax showing mass in the posterior mediastinum region
The macroscopic examination revealed a fragment of smooth tissue, firm consistence, irregular surface measuring approximately 13 cm×10 cm×5 cm. Microscopically, the lesion was consistent with the ganglioneuroma with neuronal cells in nerve fibres and calcifications in both the mass and lymph node metastases.
Postoperative patient evolution was good. However there was some difficulty expanding the lower right lobe. Six months after surgery, he was asymptomatic with normal radiology.
DISCUSSION
Median and posterior mediastinum tumours make up about 50% of all mediastinum masses in early life. The most commons are lymphomas, mediastinum cysts, and neurogenic tumours such as neuroblasts [7]. The neuroblastic tumours are the most common posterior mediastinum tumours in children [1].
Ganglioneuroma grows slowly and even with its large increase in size, half the patients had no apparent symptoms [1,4,7]. When present, symptoms are related to tumour mass or intraspinal extension [1,7]. In addition, some patients can present abdominal pain, nausea, vomiting, and thoracic pain [9]. Ganglioneuromas presenting endocrine activity are rare. Some cases can have arterial hypertension and redness [1,5].
Clinical diagnosis is by chance, while studying images taken for other clinical reasons [1,6]. Radiologically, it is a well delimited large oblong mass with a large base along the spine extending between third and fifth vertebrae. Sometimes it presents punctiform calcifications [7]. Tomography can show homogenous or heterogenous mass [4,7]. Nuclear magnetic resonance can accurately define if a tumour has intramedullar extension [2,4] as well as good correlation with histology [9].
This patient presented a tumour, which although benign, behaved aggressively invading adjacent structures and reaching nearby lymph nodes. This occurs due to the unique biological behaviour assumed to neuroblastic tumours: the possibility of spontaneous maturation [2,3,9]. It is important to keep in mind that ganglioneuroma is only a neuroblastic tumour in the final development stage [2]. Evolution to ganglioneuroma occurred spontaneously in both the tumour and the metastasis. This probably occurred when tumour was still in the neuroblastoma stage. A number of case reports has docummented maturation and spontaneous regression since the 19th century. Nevertheless, the real biological phenomena involved are not well understood [2,3,10,11]. Increasing evidence suggests that there are genetic prerequisites for the maturation process and spontaneous regression to occur including the short arm of intact chromosome 1, lack of MYCN amplification, and near-triploid DNA content [2]. Maturation and apoptosis could be induced by chemotherapy and radiotherapy, similar to therapy for neuroblastoma [3,10,12].
Taken together, this present case reports a mediastinal glanglioneuroma emphasizing further examination in children presenting upper airway infection. Therefore, pediatricians and otorhinolaryngologists should be aware on this issue.
REFERENCES
1. Forsythe A, Volpe J, Muller R. Posterior mediastinal ganglioneuroma. Radiographics, 2004,24:594-597.
2. Shimada H, Ambros IM, Dehner LP, Hata J, Joshi VV, Roald B. Terminology and morphologic criteria of neuroblastic tumors. Recommendations by the International Neuroblastoma Pathology Committee. Cancer, 1999,86:349-363.
3. Hicks MJ, Mackay B. Comparison of ultrastructural features among neuroblastic tumors: maturation from neuroblastoma to ganglioneuroma. Ultrastruct Pathol, 1995,19:311-322.
4. Scherer A, Niehues T, Engelbrecht V, M?dder U. Imaging diagnosis of retroperitoneal ganglioneuroma in childhood. Pediatr Radiol, 2001,31:106-110.
5. Tosaka A, Ando M, Arisawa C, Okano T. Endocrinologically active retroperitoneal ganglioneuroma with positive iodine-131-metaiodobenzylguanidine scintigraphy. Int J Urol,1999,6:471-474.
6. Schulman H, Laufer L, Barki Y, Philip M, Mares AJ, Maor E, Hertzanu Y. ganglioneuroma: an “incidentaloma” of chilhood. Eur Radiol,1998,8:582-584.
7. Strollo DC, Rosado-de-Christenson ML, Jett JR. Primary mediastinal tumors. Part II. Tumors of the middle and posterior mediastinum. Chest,1997,112:1344-1357.
8. Rzyman W, Skokowski J, Wilimski R, Kurowski K, Stempniewics M. One step of dumb-bell tumors by postero-lateral thoracotomy and extended foraminectomy. Eur J Cardiothorac Surg,2004,25:509-514.
9. Zhang Y, Nishimura H, Kato S, Fujimoto K, Ohkuma K, Kojima K, Uchida M, Hayabuchi N. MRI of ganglioneuroma: histologic correlation study. J Comput Assist Tomogr, 2001,25:617-623.
10. Miura K, Mineta H, Yokota N, Tsutsui Y. Olfactory neuroblastoma with epithelial and endocrine differentiation transformed into ganglioneuroma after chemoradiotherapy. Pathol Int, 2001,51:942-947.
11. Ijiri R, Tanaka Y, Kato K, Misugi K, Nishihira H, Toyoda Y, Kigasawa H, Nishi T, Takeuchi M, Aida N, Momoi T. Clinicopathologic study of mass-screened neuroblastoma with special emphasis on untreated observed cases: a possible clue to tumor regression. The American Journal of Surgery Pathology, 2000,24:807-815.
12. Berthold F, Hero B. Neuroblastoma: current drug therapy recommendations as part of the total treatment approach. Drugs,2000,59:1261-1277.
(Editor Jaque)(A. Goya1, D.C. Cataneo1, )
Correspondence to Daniel A. Ribeiro, DDS, PhD,Departamento de Patologia,Faculdade de Medicina de Botucatu-UNESP,Distrito de Rubio Jr s/n,Botucatu-SP,18618-000,Brazil
Tel: 55 14 38828255,Fax: 55 14 3815 2348,E-mail: araki@fmb.unesp.br
[Abstract] Ganglioneuroma is a benign neurogenic tumour which represents the final maturation stage of neuroblast tumours. In this case report, a mediastinal ganglioneuroma with lymph node metastasis from a five-year-old boy presenting upper airway infection is reported.
[Key words] ganglioneuroma; mediastinal neoplasms; upper airway infection; child
INTRODUCTION
Neuroblast tumours originate of precursor cells from sympathetic nervous system, consisting of the adrenal medulla and the paravertebral sympathetic ganglions [1~3]. They are composed of ganglion nerve and Schwann cells and are able to evolute from the undifferentiated and malignant neuroblastoma undergoing ganglioneuroblastoma, to mature ganglioneuroma [3], which is the most differentiated tumour in this group [1]. Classification in neuroblastoma, mixed ganglioneuroblastoma, nodular ganglioneuroblastoma, and ganglioneuroma is based on the degree of neuroblast differentiation depicted as cellular turnover index, and Schwannian stroma development. Ganglioneuromas are divided into two distinct subtypes: a) in maturation, which already presents disperse accumulation of differentiating neuroblasts, and b) mature, where the neuroblastomatose component is absent.
Ganglioneuroma is the rarest of the neuroblast tumours [4~6], where it is mainly found in the thorax (43%), particularly in the retroperitoneal space, the adrenal medulla, and the soft oropharyngeal tissues [1,4,6]. It affects the over threes, teenagers, and young adults (mean 7 years) with slight predominance in females [4,6,7]. The treatment is surgery only [8].
The goal of this current case report is to present a mediastinal ganglioneuroma with lymph node metastasis in a boy presenting upper airway infection.
CASE REPORT
A 5-year-old boy was admitted to Botucatu Medical School, Clinical Hospital (FMB-UNESP), with a clinical picture of upper airway infection. After submitting to thorax X-ray, a large asymptomatic mass with regular outline was seen occupying the inferior 1/3 of the right hemithorax, in the posterior mediastinum (Figure 1). Also, it was noted a rounded somewhat elevated area, soft to percussion and with reduced stethoscope vesicle murmur, in the lower medial right posterior thoracic region. Abdominal ultrasound showed no abdominal masses and free retroperitonium. Thoracic tomography revealed a posterior mediastinal mass with evidence of infiltration into the costal arch adjacent to the vertebrae (Figure 2). Fine needle puncture aspiration was inconclusive. Thus, an excisional biopsy was indicated by means of right posterior lateral thoracotomy. The diaphragm and median mediastinium were free. However, there was no cleavage plane between the tumour and the intercostal spaces and vertebrae. The rib which seemed to be invaded was only deformed. The cincumjacent lymph nodes were compromised (diagnosed as metastases). They were completely resected with the tumour.
Figure 1 X-ray of thorax revealing large mass with irregular forms in posterior area of mediastinum
Figure 2 Computer tomography of thorax showing mass in the posterior mediastinum region
The macroscopic examination revealed a fragment of smooth tissue, firm consistence, irregular surface measuring approximately 13 cm×10 cm×5 cm. Microscopically, the lesion was consistent with the ganglioneuroma with neuronal cells in nerve fibres and calcifications in both the mass and lymph node metastases.
Postoperative patient evolution was good. However there was some difficulty expanding the lower right lobe. Six months after surgery, he was asymptomatic with normal radiology.
DISCUSSION
Median and posterior mediastinum tumours make up about 50% of all mediastinum masses in early life. The most commons are lymphomas, mediastinum cysts, and neurogenic tumours such as neuroblasts [7]. The neuroblastic tumours are the most common posterior mediastinum tumours in children [1].
Ganglioneuroma grows slowly and even with its large increase in size, half the patients had no apparent symptoms [1,4,7]. When present, symptoms are related to tumour mass or intraspinal extension [1,7]. In addition, some patients can present abdominal pain, nausea, vomiting, and thoracic pain [9]. Ganglioneuromas presenting endocrine activity are rare. Some cases can have arterial hypertension and redness [1,5].
Clinical diagnosis is by chance, while studying images taken for other clinical reasons [1,6]. Radiologically, it is a well delimited large oblong mass with a large base along the spine extending between third and fifth vertebrae. Sometimes it presents punctiform calcifications [7]. Tomography can show homogenous or heterogenous mass [4,7]. Nuclear magnetic resonance can accurately define if a tumour has intramedullar extension [2,4] as well as good correlation with histology [9].
This patient presented a tumour, which although benign, behaved aggressively invading adjacent structures and reaching nearby lymph nodes. This occurs due to the unique biological behaviour assumed to neuroblastic tumours: the possibility of spontaneous maturation [2,3,9]. It is important to keep in mind that ganglioneuroma is only a neuroblastic tumour in the final development stage [2]. Evolution to ganglioneuroma occurred spontaneously in both the tumour and the metastasis. This probably occurred when tumour was still in the neuroblastoma stage. A number of case reports has docummented maturation and spontaneous regression since the 19th century. Nevertheless, the real biological phenomena involved are not well understood [2,3,10,11]. Increasing evidence suggests that there are genetic prerequisites for the maturation process and spontaneous regression to occur including the short arm of intact chromosome 1, lack of MYCN amplification, and near-triploid DNA content [2]. Maturation and apoptosis could be induced by chemotherapy and radiotherapy, similar to therapy for neuroblastoma [3,10,12].
Taken together, this present case reports a mediastinal glanglioneuroma emphasizing further examination in children presenting upper airway infection. Therefore, pediatricians and otorhinolaryngologists should be aware on this issue.
REFERENCES
1. Forsythe A, Volpe J, Muller R. Posterior mediastinal ganglioneuroma. Radiographics, 2004,24:594-597.
2. Shimada H, Ambros IM, Dehner LP, Hata J, Joshi VV, Roald B. Terminology and morphologic criteria of neuroblastic tumors. Recommendations by the International Neuroblastoma Pathology Committee. Cancer, 1999,86:349-363.
3. Hicks MJ, Mackay B. Comparison of ultrastructural features among neuroblastic tumors: maturation from neuroblastoma to ganglioneuroma. Ultrastruct Pathol, 1995,19:311-322.
4. Scherer A, Niehues T, Engelbrecht V, M?dder U. Imaging diagnosis of retroperitoneal ganglioneuroma in childhood. Pediatr Radiol, 2001,31:106-110.
5. Tosaka A, Ando M, Arisawa C, Okano T. Endocrinologically active retroperitoneal ganglioneuroma with positive iodine-131-metaiodobenzylguanidine scintigraphy. Int J Urol,1999,6:471-474.
6. Schulman H, Laufer L, Barki Y, Philip M, Mares AJ, Maor E, Hertzanu Y. ganglioneuroma: an “incidentaloma” of chilhood. Eur Radiol,1998,8:582-584.
7. Strollo DC, Rosado-de-Christenson ML, Jett JR. Primary mediastinal tumors. Part II. Tumors of the middle and posterior mediastinum. Chest,1997,112:1344-1357.
8. Rzyman W, Skokowski J, Wilimski R, Kurowski K, Stempniewics M. One step of dumb-bell tumors by postero-lateral thoracotomy and extended foraminectomy. Eur J Cardiothorac Surg,2004,25:509-514.
9. Zhang Y, Nishimura H, Kato S, Fujimoto K, Ohkuma K, Kojima K, Uchida M, Hayabuchi N. MRI of ganglioneuroma: histologic correlation study. J Comput Assist Tomogr, 2001,25:617-623.
10. Miura K, Mineta H, Yokota N, Tsutsui Y. Olfactory neuroblastoma with epithelial and endocrine differentiation transformed into ganglioneuroma after chemoradiotherapy. Pathol Int, 2001,51:942-947.
11. Ijiri R, Tanaka Y, Kato K, Misugi K, Nishihira H, Toyoda Y, Kigasawa H, Nishi T, Takeuchi M, Aida N, Momoi T. Clinicopathologic study of mass-screened neuroblastoma with special emphasis on untreated observed cases: a possible clue to tumor regression. The American Journal of Surgery Pathology, 2000,24:807-815.
12. Berthold F, Hero B. Neuroblastoma: current drug therapy recommendations as part of the total treatment approach. Drugs,2000,59:1261-1277.
(Editor Jaque)(A. Goya1, D.C. Cataneo1, )