Salivary Gland Neoplasms in Children David L. Callender, MD; Robert A.
Frankenthaler, MD;
Mario A. Luna,
Of 29
patients, aged 3 to 16 years, with nonvasoformasalivary gland tumors, 21 had malignant tumors. Mucoepidermoid carcinoma was the most common; adenocarcinoma, adenoid cystic carcinoma, and acinic cell carcinoma were equally represented. Pleomorphic adenoma was the exclusive benign lesion, occurring in eight patients. Mean follow-up for patients with malignant lesions \s=b\
tive
13.6 years (minimum, 3.5 years). Absolute 2- and 5-year survival rates were 100% and 90%, respectively. Mean follow-up for benign lesions was 15.9 years; none recurred. Superficial or total parotidectomy is the treatment of choice was
for malignant parotid neoplasms. Benign parotid lesions are adequately controlled with parotidectomy based on extent of disease. Facial nerve sacrifice can often be avoided. We advocate postoperative radiotherapy for high-grade lesions or those with adverse prognostic factors, such as soft-tissue extension and perineural invasion. (Arch Otolaryngol Head Neck Surg. 1992;118:472-476) of the salivary glands than of all salivary gland Neoplasms children adults1"4;
are rare in children. Fewer 5% tumors occur in and young salivary neoplasms account for less than 10% of all pediatrie head and neck tumors. The rarity of these neoplasms makes accumulation of a broad experience at one institution difficult. Between 1944 and 1987, 29 patients aged 16 years or younger who had an epithelial salivary neoplasm were seen and treated at The University of Texas M. D. Anderson Cancer Center in Houston. A similar therapeutic approach was used for all patients. A retrospective review of the results of treat¬ ment of these patients forms the basis for this study.
PATIENTS AND METHODS Between 1944 and 1987, 1822 patients with salivary gland neoplasms (574 benign and 1248 malignant) were seen at M. D. Anderson Cancer Center. Charts of the patients aged 16 years or younger who had epithelial tumors were reviewed. Patients who had hemangiomas, lymphangiomas, and sarcomas were ex¬ cluded. During the study period, 21 patients aged 16 years and younger had malignant tumors arising in the salivary glands and
Accepted From the
for
publication
December 9, 1991.
Departments of Head and Neck Surgery (Drs Callender, Frankenthaler, and Goepfert) and Pathology (Drs Luna and Lee), The University of Texas M. D. Anderson Cancer Center, Houston.
Presented at the 33rd annual meeting of the American Society for Head and Neck Surgery, Waikaloa, Hawaii, May 9, 1991. Reprint requests to the Department of Head and Neck Surgery, Box 69, The University of Texas M. D. Anderson Cancer Center, 1515 Holcombe Blvd, Houston, TX 77030 (Dr Callender).
MD; Sang Sook Lee, MD; Helmuth Goepfert, MD
eight had benign pleomorphic adenoma. The diagnosis of sali¬ gland neoplasm was confirmed in all cases by two of us (M.L. and S.L.). Mucoepidermoid carcinomas were histologically graded into grades 1 through 3 (corresponding to low, in¬ termediate, and high grade). All adenoid cystic carcinomas were classified as high-grade tumors. Other malignancies were clas¬ sified according to established criteria as low or high grade where vary
appropriate.5'10
RESULTS adenoma was the exclusive benign neo¬ Pleomorphic encountered the in 29 patients with salivary gland plasm tumors in the 16 years and younger age group; eight pa¬ tients, four boys and four girls, aged 10 to 16 years, had this benign tumor. A slowly enlarging mass was the most common initial complaint. None of the patients presented with facial nerve deficits. Six of the patients had pa¬ rotid neoplasms; two of these were located in the deep lobe. One pleomorphic adenoma was located in the sub¬ mandibular gland, and one occurred in a minor salivary gland in the mucosa overlying the hard palate. Seven of these eight patients received no prior treatment or had excisional or incisional biopsy only before referral. One patient was referred with a third recurrence after under¬ going two previous excisions. All patients in the benign group had their tumors resected at M. D. Anderson Can¬ cer Center. The mean follow-up of patients with pleo¬ morphic adenoma was 15.9 years. Minimum follow-up was 2.9 years. There were no recurrences in this group. Twenty-one patients aged 16 years and younger pre¬ sented to M. D. Anderson Cancer Center with malignant salivary gland neoplasms. The patients' ages ranged from 3 to 16 years, although only two patients were younger than 10 years (mean, 13.0 years). Nine patients in this group were boys, and 12 were girls. Thirteen patients were white, four black, and four Hispanic. The most common initial complaint of these patients was the onset of an enlarging mass in the parotid or submandibular re¬ gions. One patient with adenoid cystic carcinoma devel¬ oped facial nerve weakness and pain shortly after onset of a parotid mass. Four patients with mucoepidermoid carcinoma of the parotid presented with palpable cervical adenopathy that was pathologically benign. Mucoepidermoid carcinoma was the most common tu¬ mor in the 21 patients with salivary gland malignancies. The parotid gland was the primary site of mucoepider¬ moid carcinoma in 10 cases, whereas two cases occurred in submandibular glands. Three acinic cell carcinomas and three adenocarcinomas arose in parotid glands. Ad-
Downloaded From: http://archotol.jamanetwork.com/ by a UQ Library User on 06/16/2015
eight
•
· · ·
-·-·-
I
10
15 Years
—r-
~ -1—
20
25
30
10
15 Years
20
25
30
Fig 1 .—Cumulative survival rates in children with malignant salivary gland neoplasms.
Fig 2. Cumulative control rates in children with malignant salivary gland neoplasms receiving primary treatment.
cystic carcinoma occurred in the parotid gland of patients and in the submandibular gland of one pa¬ tient. Overall, 18 malignant neoplasms occurred in the parotid gland and three in the submandibular gland. Fourteen of the 21 patients with salivary gland cancer had received no treatment or had undergone only incisional or excisional biopsy of their tumor before referral. These 14 patients received definitive treatment at M. D. Anderson Cancer Center. Two additional patients re¬ ceived radiotherapy as adjunctive treatment at M. D. Anderson Cancer Center following surgical resection of their tumors at other institutions. The remaining five pa¬
tients
enoid two
recurrent cancer, underwent salvage procedures at our institution. Following treatment at our institution, 17 of 21 patients with malignancy remained free of disease for a minimum of 3.5 years anda maximum of30.5years(mean follow-up of 13.6 years). Two patients were living with disease at 20.2 and 7.8 years of follow-up. Two patients died of dis¬ ease. Absolute 2- and 5-year survival rates were 1007c and 90%, respectively (Fig 1). Of the 14 patients who underwent definitive treatment
tients, who had
at M. D. Anderson Cancer Center, 13 are disease free at 6.8 to 30.5 years of follow-up. One patient is living with disease at 7.8 years of follow-up (Fig 2). Thirteen of the 14
patients in this group had parotid malignancies and one had a submandibular malignancy. All of the 14 patients underwent surgical procedures and one of them received postoperative radiotherapy. For parotid malignancies, a parotidectomy that provided an adequate tumor margin was performed. For submandibular tumors, regional dis¬ section of the submandibular triangle was performed. Upper neck dissection was performed with each paroti¬ dectomy. Modified neck dissections were performed for high-grade tumors and for clinically suspicious adenopathy. Two patients received radiotherapy at our institution as additional treatment after having surgical resections of their tumor elsewhere. One of these patients had adenoid cystic carcinoma of the submandibular gland and one had a high-grade adenocarcinoma of the parotid gland. Both are free of disease at 14.0 and 18.2 years of follow-up, re¬ spectively. Five patients with salivary malignancies were referred with recurrent tumors and underwent salvage surgery at M. D. Anderson Cancer Center. All had had surgical re¬ section and radiotherapy elsewhere. Two of the five pa-
—
are disease free 3.5 and 6.0 years after salvage sur¬ One patient is living with disease at 20.2 years of gery. follow-up, and two died of disease 3.3 and 5.8 years after
salvage treatment (Table). Overall, four
tient who failed
treatment failures occurred. The one pa¬ primary treatment at this institution had
aggressive adenoid cystic carcinoma of the parotid gland. The patient underwent total parotidectomy with
an
facial nerve resection and modified neck dissection, and received postoperative radiotherapy. Despite locore¬ gional control, the patient developed distant metastasis 7.1 years following initial treatment. Of the five surgical salvage procedures performed at M. D. Anderson Cancer Center, three were not success¬ ful. All of the five patients presented to M. D. Anderson Cancer Center with local recurrences, and two had regional recurrences as well. The three patients whose salvage procedures failed all had high-grade parotid can¬ cers. One patient had recurrences at local, regional, and distant sites within 1 year of the attempted salvage pro¬ cedure. The other two patients who had salvage failures had recurrences at local and distant sites 1.3 and 10.4 years after their procedures. A total of four patients underwent resection of the main trunk of the facial nerve. Two of the four patients had fa¬ cial nerve invasion at the time of the original surgical therapy and underwent main trunk resection. The other two patients presented with recurrent disease with ex¬ tension into the nerve and had nerve sacrifice during sal¬ vage procedures. Both of the patients who had main trunk resection during a salvage procedure are dead of disease. One of the patients who had nerve sacrifice during primary surgery at M. D. Anderson Cancer Center is liv¬ ing with disease. The remaining patient who underwent main trunk resection is alive with no evidence of disease. Six patients had resection of one to three branches of the facial nerve for complete tumor excision. Two of the six underwent nerve grafting. Five of the six have no clinical deficit, whereas one has paresis of the marginal branch. All six are alive with no evidence of disease. Of the 18 pa¬ tients with malignant parotid tumors who had a surgical procedure, eight had no nerve resection and have normal function after surgery. Five patients returned to satisfac¬ tory function following resection and recovery. All patients who had parotid tumors and who had ini¬ tial surgery at this institution had upper neck dissection for examination of surrounding local lymph nodes. Upper
Downloaded From: http://archotol.jamanetwork.com/ by a UQ Library User on 06/16/2015
. ·
Status of Patients After
Salvage Surgery for Malignancy*
Tumor
Location
cystic Mucoepidermoid
Disease Status
Follow"P> y
0.8o
Adenoid
Parotid
LWD
20.2
DOD
5.8
Acinic cell
Parotid Parotid
DOD
3.3
E
Parotid Submandibular
NED
6.0
U
NED
3.5
Adenocarcinoma
Mucoepidermoid
*NED indicates disease free; LWD, dead of disease.
0.6· 0.4
0.2
living with disease; and DOD,
No Factors (n=13) !>1 Factor (n=8) P=.001
0.0
10
r··-^
1.0
-·-·-
-·-·-
-·-·-
0.8-
Frankenthaler, MD;
Mario A. Luna,
Of 29
patients, aged 3 to 16 years, with nonvasoformasalivary gland tumors, 21 had malignant tumors. Mucoepidermoid carcinoma was the most common; adenocarcinoma, adenoid cystic carcinoma, and acinic cell carcinoma were equally represented. Pleomorphic adenoma was the exclusive benign lesion, occurring in eight patients. Mean follow-up for patients with malignant lesions \s=b\
tive
13.6 years (minimum, 3.5 years). Absolute 2- and 5-year survival rates were 100% and 90%, respectively. Mean follow-up for benign lesions was 15.9 years; none recurred. Superficial or total parotidectomy is the treatment of choice was
for malignant parotid neoplasms. Benign parotid lesions are adequately controlled with parotidectomy based on extent of disease. Facial nerve sacrifice can often be avoided. We advocate postoperative radiotherapy for high-grade lesions or those with adverse prognostic factors, such as soft-tissue extension and perineural invasion. (Arch Otolaryngol Head Neck Surg. 1992;118:472-476) of the salivary glands than of all salivary gland Neoplasms children adults1"4;
are rare in children. Fewer 5% tumors occur in and young salivary neoplasms account for less than 10% of all pediatrie head and neck tumors. The rarity of these neoplasms makes accumulation of a broad experience at one institution difficult. Between 1944 and 1987, 29 patients aged 16 years or younger who had an epithelial salivary neoplasm were seen and treated at The University of Texas M. D. Anderson Cancer Center in Houston. A similar therapeutic approach was used for all patients. A retrospective review of the results of treat¬ ment of these patients forms the basis for this study.
PATIENTS AND METHODS Between 1944 and 1987, 1822 patients with salivary gland neoplasms (574 benign and 1248 malignant) were seen at M. D. Anderson Cancer Center. Charts of the patients aged 16 years or younger who had epithelial tumors were reviewed. Patients who had hemangiomas, lymphangiomas, and sarcomas were ex¬ cluded. During the study period, 21 patients aged 16 years and younger had malignant tumors arising in the salivary glands and
Accepted From the
for
publication
December 9, 1991.
Departments of Head and Neck Surgery (Drs Callender, Frankenthaler, and Goepfert) and Pathology (Drs Luna and Lee), The University of Texas M. D. Anderson Cancer Center, Houston.
Presented at the 33rd annual meeting of the American Society for Head and Neck Surgery, Waikaloa, Hawaii, May 9, 1991. Reprint requests to the Department of Head and Neck Surgery, Box 69, The University of Texas M. D. Anderson Cancer Center, 1515 Holcombe Blvd, Houston, TX 77030 (Dr Callender).
MD; Sang Sook Lee, MD; Helmuth Goepfert, MD
eight had benign pleomorphic adenoma. The diagnosis of sali¬ gland neoplasm was confirmed in all cases by two of us (M.L. and S.L.). Mucoepidermoid carcinomas were histologically graded into grades 1 through 3 (corresponding to low, in¬ termediate, and high grade). All adenoid cystic carcinomas were classified as high-grade tumors. Other malignancies were clas¬ sified according to established criteria as low or high grade where vary
appropriate.5'10
RESULTS adenoma was the exclusive benign neo¬ Pleomorphic encountered the in 29 patients with salivary gland plasm tumors in the 16 years and younger age group; eight pa¬ tients, four boys and four girls, aged 10 to 16 years, had this benign tumor. A slowly enlarging mass was the most common initial complaint. None of the patients presented with facial nerve deficits. Six of the patients had pa¬ rotid neoplasms; two of these were located in the deep lobe. One pleomorphic adenoma was located in the sub¬ mandibular gland, and one occurred in a minor salivary gland in the mucosa overlying the hard palate. Seven of these eight patients received no prior treatment or had excisional or incisional biopsy only before referral. One patient was referred with a third recurrence after under¬ going two previous excisions. All patients in the benign group had their tumors resected at M. D. Anderson Can¬ cer Center. The mean follow-up of patients with pleo¬ morphic adenoma was 15.9 years. Minimum follow-up was 2.9 years. There were no recurrences in this group. Twenty-one patients aged 16 years and younger pre¬ sented to M. D. Anderson Cancer Center with malignant salivary gland neoplasms. The patients' ages ranged from 3 to 16 years, although only two patients were younger than 10 years (mean, 13.0 years). Nine patients in this group were boys, and 12 were girls. Thirteen patients were white, four black, and four Hispanic. The most common initial complaint of these patients was the onset of an enlarging mass in the parotid or submandibular re¬ gions. One patient with adenoid cystic carcinoma devel¬ oped facial nerve weakness and pain shortly after onset of a parotid mass. Four patients with mucoepidermoid carcinoma of the parotid presented with palpable cervical adenopathy that was pathologically benign. Mucoepidermoid carcinoma was the most common tu¬ mor in the 21 patients with salivary gland malignancies. The parotid gland was the primary site of mucoepider¬ moid carcinoma in 10 cases, whereas two cases occurred in submandibular glands. Three acinic cell carcinomas and three adenocarcinomas arose in parotid glands. Ad-
Downloaded From: http://archotol.jamanetwork.com/ by a UQ Library User on 06/16/2015
eight
•
· · ·
-·-·-
I
10
15 Years
—r-
~ -1—
20
25
30
10
15 Years
20
25
30
Fig 1 .—Cumulative survival rates in children with malignant salivary gland neoplasms.
Fig 2. Cumulative control rates in children with malignant salivary gland neoplasms receiving primary treatment.
cystic carcinoma occurred in the parotid gland of patients and in the submandibular gland of one pa¬ tient. Overall, 18 malignant neoplasms occurred in the parotid gland and three in the submandibular gland. Fourteen of the 21 patients with salivary gland cancer had received no treatment or had undergone only incisional or excisional biopsy of their tumor before referral. These 14 patients received definitive treatment at M. D. Anderson Cancer Center. Two additional patients re¬ ceived radiotherapy as adjunctive treatment at M. D. Anderson Cancer Center following surgical resection of their tumors at other institutions. The remaining five pa¬
tients
enoid two
recurrent cancer, underwent salvage procedures at our institution. Following treatment at our institution, 17 of 21 patients with malignancy remained free of disease for a minimum of 3.5 years anda maximum of30.5years(mean follow-up of 13.6 years). Two patients were living with disease at 20.2 and 7.8 years of follow-up. Two patients died of dis¬ ease. Absolute 2- and 5-year survival rates were 1007c and 90%, respectively (Fig 1). Of the 14 patients who underwent definitive treatment
tients, who had
at M. D. Anderson Cancer Center, 13 are disease free at 6.8 to 30.5 years of follow-up. One patient is living with disease at 7.8 years of follow-up (Fig 2). Thirteen of the 14
patients in this group had parotid malignancies and one had a submandibular malignancy. All of the 14 patients underwent surgical procedures and one of them received postoperative radiotherapy. For parotid malignancies, a parotidectomy that provided an adequate tumor margin was performed. For submandibular tumors, regional dis¬ section of the submandibular triangle was performed. Upper neck dissection was performed with each paroti¬ dectomy. Modified neck dissections were performed for high-grade tumors and for clinically suspicious adenopathy. Two patients received radiotherapy at our institution as additional treatment after having surgical resections of their tumor elsewhere. One of these patients had adenoid cystic carcinoma of the submandibular gland and one had a high-grade adenocarcinoma of the parotid gland. Both are free of disease at 14.0 and 18.2 years of follow-up, re¬ spectively. Five patients with salivary malignancies were referred with recurrent tumors and underwent salvage surgery at M. D. Anderson Cancer Center. All had had surgical re¬ section and radiotherapy elsewhere. Two of the five pa-
—
are disease free 3.5 and 6.0 years after salvage sur¬ One patient is living with disease at 20.2 years of gery. follow-up, and two died of disease 3.3 and 5.8 years after
salvage treatment (Table). Overall, four
tient who failed
treatment failures occurred. The one pa¬ primary treatment at this institution had
aggressive adenoid cystic carcinoma of the parotid gland. The patient underwent total parotidectomy with
an
facial nerve resection and modified neck dissection, and received postoperative radiotherapy. Despite locore¬ gional control, the patient developed distant metastasis 7.1 years following initial treatment. Of the five surgical salvage procedures performed at M. D. Anderson Cancer Center, three were not success¬ ful. All of the five patients presented to M. D. Anderson Cancer Center with local recurrences, and two had regional recurrences as well. The three patients whose salvage procedures failed all had high-grade parotid can¬ cers. One patient had recurrences at local, regional, and distant sites within 1 year of the attempted salvage pro¬ cedure. The other two patients who had salvage failures had recurrences at local and distant sites 1.3 and 10.4 years after their procedures. A total of four patients underwent resection of the main trunk of the facial nerve. Two of the four patients had fa¬ cial nerve invasion at the time of the original surgical therapy and underwent main trunk resection. The other two patients presented with recurrent disease with ex¬ tension into the nerve and had nerve sacrifice during sal¬ vage procedures. Both of the patients who had main trunk resection during a salvage procedure are dead of disease. One of the patients who had nerve sacrifice during primary surgery at M. D. Anderson Cancer Center is liv¬ ing with disease. The remaining patient who underwent main trunk resection is alive with no evidence of disease. Six patients had resection of one to three branches of the facial nerve for complete tumor excision. Two of the six underwent nerve grafting. Five of the six have no clinical deficit, whereas one has paresis of the marginal branch. All six are alive with no evidence of disease. Of the 18 pa¬ tients with malignant parotid tumors who had a surgical procedure, eight had no nerve resection and have normal function after surgery. Five patients returned to satisfac¬ tory function following resection and recovery. All patients who had parotid tumors and who had ini¬ tial surgery at this institution had upper neck dissection for examination of surrounding local lymph nodes. Upper
Downloaded From: http://archotol.jamanetwork.com/ by a UQ Library User on 06/16/2015
. ·
Status of Patients After
Salvage Surgery for Malignancy*
Tumor
Location
cystic Mucoepidermoid
Disease Status
Follow"P> y
0.8o
Adenoid
Parotid
LWD
20.2
DOD
5.8
Acinic cell
Parotid Parotid
DOD
3.3
E
Parotid Submandibular
NED
6.0
U
NED
3.5
Adenocarcinoma
Mucoepidermoid
*NED indicates disease free; LWD, dead of disease.
0.6· 0.4
0.2
living with disease; and DOD,
No Factors (n=13) !>1 Factor (n=8) P=.001
0.0
10
r··-^
1.0
-·-·-
-·-·-
-·-·-
0.8-
