Low-grade papillary adenocarcinoma of minor salivary glands Case report and literature review Reza MostoJi, DMD, MS, Robert S. Wood, DOS, William Aleksander Talerman, MD, PhD, Chicago, Ill. DEPARTMENTS
OF PATHOLOGY
AND
SURGERY,
UNIVERSITY
Christison,
DDS, MS, and
OF CHICAGO
Low-grade papillary adenocarcinoma of minor salivary glands is rare and tends to occur in the palate. This tumor has distinct but rather deceptive histomorphologic features, which may cause erroneous diagnosis. An extensive English-language literature review revealed reports of 22 well-documented low-grade papillary adenocarcinomas. Six of these tumors (27%) recurred locally between 12 months and 19 years after initial treatment. Low-grade papillary adenocarcinoma appears to have more aggressive biologic behavior compared with other low-grade adenocarcinomas in this region. Four of the reported cases (17%) had cervical lymph node metastases at the time of Ipresentation. VVe report an additional case and discuss the literature. (ORAL
SURC
ORAL
MED
ORAL
PATHOL
1992;73;591-5)
P
apillary tumors of minor salivary glands are rare, and most of these lesions are described in the literature as papillary cystadenoma.’ Whittaker and Turner’ divided papillary lesions of the minor salivary glands into three groups and reported two examples of each. These were focal adenomatoid hyperplasia, papillary adenoma, and papillary carcinoma. In addition to the tumors with predominant papillary features, other salivary gland neoplasms may contain some papillary areas. These tumors include benign mixed tumor, adenocystic carcinoma, acinic cell adenocarcinoma, and polymorphous low-grade adenocarcinoma. Low-grade papillary adenocarcinoma (LGPA) of the minor salivary glands is histologically and clinically a distinct neoplasm with a marked predilection for the palate. Because of their rare occurrence and deceptive histopathologic features, these tumors are often misdiagnosed.2l 3 LGPA reportedly involves more males than females, with a 2:l ratio, and is more common after 40 years of age. We recently encountered a typical case. In view of the rare occurrence and the diagnostic problems caused by this entity, the case is reported. CASE
REPORT
A 65year-old black man was referred to one of the authors (R.S.W.) by his dentist for evaluation and treatment 7114129125
Fig.
1. Tumor
at junction
of right soft and hard palate.
of a swelling at the junction of the hard and soft palate. The patient related that the swelling was asymptomatic. The lesion had been present for approximately 11 months and had slowly increased in size. The patient’s medical history was noncontributory. An extraoral examination was negative for swelling, tenderness, or lymphadenopathy. Intraoral examination revealed edentulous jaws with generally normal-appearing mucosa throughout. In the region of the right lateral posterior palate, a 2 X 2 cm swelling was noted (Fig. 1). The overlying mucosa appeared to be pale blue and normal in texture. The swelling was moderately firm and slightly tender. Conventional panoramic and occlusal radiographic examinations did not reveal osseous lesions or other abnormalities. Computed tomographic examination, however, 591
992
Mostoj
et al.
ig. 2. Photomicrograph showing cystic spaces of different sizes in mass of tumor adjacent to minor salivary gland. (Hemotoxylin-eosin stain; original magnification, X40.)
ig. 4. Photomicrograph showing tumor cells with granular cytoplasm and intraluminal psammoma body. (Hemotoxylin-eosin stain; original magnification, X400.)
Table
I. Results Antibody
Keratin
S- 100 protein Vimentin Desmin CEA (polyclonal)
CEA CEA,
Fig. 3. A, and B, Photomicrographs showing cystic spaces lined by columnar epithelial cells in complex papillary configurations. (Hemotoxylin-eosin stain; original magnifications: X 100.) showed a 2 X 1 X 1 cm lobulated mass with peripheral enhancement and mild alveolar bone erosion. Aspiration of the lesion before incisional biopsy produced 0.5 ml of dark mutinous material. Histopathologic examination of the biopsy material revealed unremarkable mucosa. Deep in the connective tissue were several cystic spaces of various size lined by epithelium, forming intraluminal
(monoclonal) Carcinoembryonic
of immunohistochemical
I
Adjacent salivary
minor gland
studies 1
Tumor
Duct cells strongly + Myoepithelial cells + -
Strongly Strongly -
Luminal cell membrane of acinic cells and ductal cells weakly + -
-I-
+ +
-
antigen
papillary projections (Fig. 2). The cells of the epithelial lining were columnar, with eosinophilic, slightly granular, and occasionally clear cytoplasm. There was moderate nuclear pleomorphism with prominent nucleoli and a low mitotic index (Fig. 3). Occasionally, intraluminal psammoma bodies were present (Fig. 4). The cytoplasm of the tumor cells appeared to be slightly periodic acid-Schiff positive (diastase failed to change the staining quality), whereas mucicarmine stain was negative for intracytoplasmic mutin. Immunocytochemical studies were performed with the avidin-biotin-peroxidase technique.4 The following primary antibodies were used: keratins (CAM 5.2, monoclonal; Becton Dickinson & Co., Mountain View, Calif.), AEi/ AE3 (monoclonal; Hybritech, San Diego, Calif.), S-100 protein (polyclonal; Dako Corp., Santa Barbara, Calif.); vimentin (monoclonal; Dako), desmin (monoclonal; Amersham International Ltd., Little Chalfont, U.K.), and carcinoembryonic antigen (polyclonal and monoclonal; Dako). The results of this study are summarized in Table I. Tumor cells reacted for keratin, S-100 protein, and polyclonal carcinoembryonic antigen. A diagnosis of papillary adenocarcinoma, possibly of salivary gland origin, was made. Preoperative evaluation (including complete physical examination, chest x-ray fiim,
Volume Number
Low-grade
73 5
Table II. Reported cases of LGPA
Reported duration M/65
adenocarcinoma
593
of minor salivary glands
Author EdwardsI
papillary
Hard
palate
Follow-up
Treatment
-
Excision
No recurrence
after
1 yr Calhoun et aL6 Cady and Huttert3 Brooks et a1.5 Case 1 Case 2 Whittaker and Turner’ Case 5
Case 6
Mills
et al.?
F/51
Upper
M/48
Palate alveolar ridge
Excision
M/31
Hard
Excision
M/63
Soft palate
6 yr
Excision
F/53
Hard and soft palate
3 yr
Excision
M/56
Buccal
6 mo
Excision, lymph node dissection
Shteyer and FundoianuDayart’ Slootweg and Muller”
Recurrence
after
43
yr
palate
No recurrence 22 mo
after
F/48
Hard
palate
Several
M/50
Hard
palate
8-10 yr
Excision
M/30
Hard and soft palate Hard and soft palate Hard and soft palate Sublingual submandibular lymph node Right midface
3-4 yr
Wide excision
20 yr
Wide
No recurrence
M/42 et a1.t4
10 days (?)
Recurrence after 6 yr; reexcision; 2nd recurrence 1 yr later; radiation and reexcision (5000 rad) Recurrence after 8 yr; reexcision and radiation Recurrence after 19 yr followed by cervical lymph node metastasis Recurrence after 1 yr with cervical lymph node metastasis No recurrence 10 yr
F/71
Cracker
lip
M/5
M/71 M/76
mucosa
Soft palate
mo
Excision
excision
9 mo
Wide
3 mo (?)
Hemiglossectomy and modified neck dissection Radiation
1 mo (?) 1 wk-I
yr
excision
Resection
after
9 Yr Cervical metastasis after 7 yr No recurrence after
2 Yr No recurrence
after
3 yr No recurrence
after
2 Yr
TX, Treatment
M/74
Hard
palate
1 wk-7
yr
Excision
M/56
Hard
palate
1 wk-7
yr
Recurrence followed by electrocautery
M/52
Hard palate, submandibular
1 wk-7
yr
Resection and excision of lymph node
No recurrence after 14 yr Recurrence after 18 yr; reexcision; 2nd recurrence after 1 yr; reexcision Recurrent lymph node metastasis in neck, 3 recurrences at 1, 5, and 11 yr; final TX was neck dissection 12 yr after 1st TX; metastasis in lung and vertebra; died of tumor 20 yr after initial TX
Mostoji et al.
594
ORAL
%JKG
ORAL
MED
ORAL
?ATti0~
May
Table
1992
II. Cont’d. Treatment
F/61
study
palate
1 wk-I
yr
1 wk-7
F/42
1 wk-I
yr
Resection
F/87
Soft palate
1 wk-7
yr
M/65
Hard
palate
11 yr
yr
Resection
Hard palate, cervical lymph node Hard palate
M/60
Present
Hard
Resection, neck dissection
Follow-up No recurrence
after
9 Yr No recurrence
after
2 Yr
Resection
No recurrence 25 yr No recurrence
after after
Resection
1 yr No recurrence
after
3 Yr
bonescan,completebloodcell count, andserumchemistry) wasnegativefor metastasis andorganinvolvement.The remainingtumor wasremovedby hemimaxillectomy,through a modifiedWeber-Fergusson incision.The patient tolerated the procedurewell, andthe defectwaseventuallyobtunded with a prostheticappliance.The patient is alive and free of disease3 years after surgery. DISCUSSION According to the World Health Organization salivary gland tumor classification, tumors with a papillary pattern fall into the group of adenocarcinoma. More than 75% of the papillary, mutinous, and other unusual variants of adenocarcinoma arise in minor salivary glands. Allen et a1.sreviewed 67 casesof primary tumors of the palate. Only two tumors were papillary adenocarcinomas, which were designated LGPA. LGPA of salivary gland origin is a rare tumor with high predilection for the oral cavity in genera! and the palate in particular. This tumor has been reported in the literature under a variety of names, including papillary cystadenoma, papillary cystadenocarcinoma, and papillary-cystic adenocarcinoma.‘, 5-10An exhaustive English-language literature review revealed only 22 well-documented cases1-3T 5-14Nine casesin a seriesof 492 casesof minor salivary gland tumors reviewed by Spiro et a1.7were papillary adenocarcinomas. They labeled these tumors papillarycystic adenocarcinomas. Evans and Batsakis,15however, argued that the casesreported by Allen et a1.3 and Spiro et ah7 were polymorphous low-grade adenocarcinomas. Slootweg and Muller”’ recently reviewed 15 cases of low-grade adenocarcinomas of the oral cavity. Seven of these caseshad features similar to terminal duct adenocarcinoma, a subtype of polymorphous low-grade adenocarcinoma, and eight were LGPA.
They concluded that terminal duct adenocarcinoma and LGPA show sufficient differences, both histologically and clinically, to be considered as separate entities. Because of its rarity and its deceptively bland histologic appearance, LGPA isoften misdiagnosed.The only way to distinguish LGPA from other minor salivary gland neoplasmsin this region is by meansof a careful histopathologic examination. A major feature of the LGPA is the presence of papillary proliferations that project into cystlike spaces.The tumor cells are either cuboidal or columnar and are arranged in papillary configurations that vary from simple to complex.2 Occasional mitotic figures and moderate nuclear pleomorphism are present. Several benign and malignant lesions should be considered in the differential diagnosis.These lesions include mucopapillary cyst,16 adenomatous papillary hyperplasia, papillary cystadenoma, benign mixed tumor, adenocystic carcinoma, mucoepidermoid carcinoma, acinic cell adenocarcinoma, and polymorphous low-grade adenocarcinoma. Benign salivary tumors in this region (papillary cystadenoma in particular) could readily be differentiated from LGPA. LGPA is an unencapsulated and infiltrative tumor that exhibits somecellular pleomorphism. Other mahgnant salivary lesions, however, have characteristic histopathologic features that distinguish them from LGPA. Clinically, LGPA is a slow-growing neoplasm(Table 11). The agesof the patients in the reported cases ranged from 5 to 87 years (mean 54.7 years). Of these reported cases,15 patients were male and 7 were female. In all the reported casesLGPA originated in the minor salivary glands, predominantly in the palate (posterior; either hard or soft). The size of most tumors at the time of clinical evaluation varied from 1.5 to 3 cm. The tumors were usually covered by in-
Volume Number
Low-grade papillary adenocarcinoma 595
73 5
tact mucosa. Swelling was the most common clinical presentation, whereas occasionally ulceration and pain were additional clinical symptoms. Treatment of choice for LGPA is wide local excision, including soft tissue and bone.17 There is a tendency for local recurrence if the tumor has not been excised completely. Six of 22 reported tumors (27%) recurred locally. Local recurrence in these cases appeared to be unrelated to the duration of sym!ptoms and size of the tumor. Disease-free intervals between local recurrences in these cases varied from 12 months to 19 years. In comparison with other types of low-grade adenocarcinoma of minor salivary glands in this region, LGPA appears to have a greater tendency to metastasize. In four (17%) of the reported cases cervical lymph node metastases were noted at the time of presentation. The present case represents a classic examlple of LGPA both clinically and histopathologically. Because of the radiographic report of possible bone involvement and the patient’s age, hemimaxillectomy was considered the tl:eatment of choice by the members of the tumor board. REFERENCES 1. Whittaker JS, Turner EP. Papillary tumors of the minor salivary glands. J Clin Path01 1976;29:‘795-805. 2. Mills SE, Garland TA, Allen MS. Low-grade papillary adenocarcinoma of the palatal salivary gland origin. Am J Surg Path01 1984;8:367-74. 3. Allen MS, Fitz-Hugh GS, Marsh WL. Low-grade papillary adenocarcinoma of the palate. Cancer 1974;33:153-8. 4. Hsu SM, Raine L, Fanger H. Use of avidin-biotin-peroxidase complex (ABC) in immunoperoxidase techniques: a compar-
ison between ABC and unlabelled antibody (PAP) procedures. J Histochem Cytochem 1981;29:577-80. 5. Brooks HW, Hiebert AE, Pullman NK, Stofer BE. Papillary cystadenoma of the palate: a review of the literature and report of two new cases. ORAL SURC ORAL MED ORAL PATHOL
1956;9:1047-50.
6. Calhoun NR, Cerine FC, Mathews MJ. Papillary cystadenoma of the upper lip; report of a case. ORAL SURGORAL MED
ORAL PATHOL 1965;20:810-3. 7. Spiro RH, Koss LG, Strong EW. Tumors of minor salivary origin: a clinicopathologic study of 492 cases. Cancer 1973; 31:117-29. 8. Spiro RH, Huvos AG, Strong EW. Adenocarcinoma of salivary origin: clinicopathologic study of 204 patients. Am J Surg 1982;144:423-31. 9. Blanck C, Eneroth CM, Jakobsson PA. Mucus-producing adenopapillary (non-epidermoid) carcinoma of the parotid gland. Cancer 1971;28:676-85. 10. Shteyer A, Fundoianu-Dayan D. Papillary cystic adenocarcinoma of minor salivary glands. Int J Oral Maxillofac Surg 1986;15:361-4. 11. Slootweg PJ, Muller H. Low-grade adenocarcinoma of theoral cavity: a comparison between the terminal duct and papillary type. J Craniomaxillofac Surg 1987;15:359-64. 12. Edwards EG. Tumors of the minor salivary glands: a report of 23 cases. Am J Clin Path01 1960;34:455-63. 13. Cady B, Hutter RVP. Non-epidermoid cancer of the gum. Cancer 1969;23:1318-24. 14. Cracker TP, Kreutner A, Othersen HB, Garvin AJ. Papillary adenocarcinoma of minor salivary gland origin in a child. Arch Otolaryngol 1983;109:827-31. 15. Evans HL, Batsakis JG. Polymorphous low-grade adenocarcinoma of minor salivary glands: a study of 14 cases of a distinctive neoplasm. Cancer 1984;53:935-42. 16. Eversole LR. Oral sialocysts. Arch Otolaryngol Head Neck Surg 1987;113:51-6. 17. Stuteville OH, Corley RD. Surgical management of tumors of intraoral minor salivary glands. Cancer 1967;20:1578-86. Reprint requests: Reza Mostofi, DMD, 3403 W. Bryn Mawr Chicago, IL 60659
MS
BOUND VOLUMES AVAILABLE TO SUBSCRIBERS Boundvolumesof ORALSURGERY,ORAL MEDICINE,AND ORALPATHOLOGY areavailableto subscribers (only) for the 199 1 issues from the Publisher, at a cost of $45.00 for domestic, $60.15 for Canadian, and $57.00 for international, for Vol. 71 (January-June) and Vol. 72 (July-December). Shipping charges are included. Each bound volume contains a subject and author index and all advertising is removed. Copies are shipped within 60 days after publication of the last issue in the volume. The binding is durable buckram with the journal name, volume number, and year stamped in gold on the spine. Payment must accompany all orders. Contact Mosby-Year Book, Inc., Subscription Services, 11830 Westline Industrial Drive, St. Louis, MO 63146-3318, USA; phone (800)325-4177, ext. 4351, or (314)453-4351. Subscriptions must be in force to qualify. Bound volumes are not available in place of a regular journal subscription.
OF PATHOLOGY
AND
SURGERY,
UNIVERSITY
Christison,
DDS, MS, and
OF CHICAGO
Low-grade papillary adenocarcinoma of minor salivary glands is rare and tends to occur in the palate. This tumor has distinct but rather deceptive histomorphologic features, which may cause erroneous diagnosis. An extensive English-language literature review revealed reports of 22 well-documented low-grade papillary adenocarcinomas. Six of these tumors (27%) recurred locally between 12 months and 19 years after initial treatment. Low-grade papillary adenocarcinoma appears to have more aggressive biologic behavior compared with other low-grade adenocarcinomas in this region. Four of the reported cases (17%) had cervical lymph node metastases at the time of Ipresentation. VVe report an additional case and discuss the literature. (ORAL
SURC
ORAL
MED
ORAL
PATHOL
1992;73;591-5)
P
apillary tumors of minor salivary glands are rare, and most of these lesions are described in the literature as papillary cystadenoma.’ Whittaker and Turner’ divided papillary lesions of the minor salivary glands into three groups and reported two examples of each. These were focal adenomatoid hyperplasia, papillary adenoma, and papillary carcinoma. In addition to the tumors with predominant papillary features, other salivary gland neoplasms may contain some papillary areas. These tumors include benign mixed tumor, adenocystic carcinoma, acinic cell adenocarcinoma, and polymorphous low-grade adenocarcinoma. Low-grade papillary adenocarcinoma (LGPA) of the minor salivary glands is histologically and clinically a distinct neoplasm with a marked predilection for the palate. Because of their rare occurrence and deceptive histopathologic features, these tumors are often misdiagnosed.2l 3 LGPA reportedly involves more males than females, with a 2:l ratio, and is more common after 40 years of age. We recently encountered a typical case. In view of the rare occurrence and the diagnostic problems caused by this entity, the case is reported. CASE
REPORT
A 65year-old black man was referred to one of the authors (R.S.W.) by his dentist for evaluation and treatment 7114129125
Fig.
1. Tumor
at junction
of right soft and hard palate.
of a swelling at the junction of the hard and soft palate. The patient related that the swelling was asymptomatic. The lesion had been present for approximately 11 months and had slowly increased in size. The patient’s medical history was noncontributory. An extraoral examination was negative for swelling, tenderness, or lymphadenopathy. Intraoral examination revealed edentulous jaws with generally normal-appearing mucosa throughout. In the region of the right lateral posterior palate, a 2 X 2 cm swelling was noted (Fig. 1). The overlying mucosa appeared to be pale blue and normal in texture. The swelling was moderately firm and slightly tender. Conventional panoramic and occlusal radiographic examinations did not reveal osseous lesions or other abnormalities. Computed tomographic examination, however, 591
992
Mostoj
et al.
ig. 2. Photomicrograph showing cystic spaces of different sizes in mass of tumor adjacent to minor salivary gland. (Hemotoxylin-eosin stain; original magnification, X40.)
ig. 4. Photomicrograph showing tumor cells with granular cytoplasm and intraluminal psammoma body. (Hemotoxylin-eosin stain; original magnification, X400.)
Table
I. Results Antibody
Keratin
S- 100 protein Vimentin Desmin CEA (polyclonal)
CEA CEA,
Fig. 3. A, and B, Photomicrographs showing cystic spaces lined by columnar epithelial cells in complex papillary configurations. (Hemotoxylin-eosin stain; original magnifications: X 100.) showed a 2 X 1 X 1 cm lobulated mass with peripheral enhancement and mild alveolar bone erosion. Aspiration of the lesion before incisional biopsy produced 0.5 ml of dark mutinous material. Histopathologic examination of the biopsy material revealed unremarkable mucosa. Deep in the connective tissue were several cystic spaces of various size lined by epithelium, forming intraluminal
(monoclonal) Carcinoembryonic
of immunohistochemical
I
Adjacent salivary
minor gland
studies 1
Tumor
Duct cells strongly + Myoepithelial cells + -
Strongly Strongly -
Luminal cell membrane of acinic cells and ductal cells weakly + -
-I-
+ +
-
antigen
papillary projections (Fig. 2). The cells of the epithelial lining were columnar, with eosinophilic, slightly granular, and occasionally clear cytoplasm. There was moderate nuclear pleomorphism with prominent nucleoli and a low mitotic index (Fig. 3). Occasionally, intraluminal psammoma bodies were present (Fig. 4). The cytoplasm of the tumor cells appeared to be slightly periodic acid-Schiff positive (diastase failed to change the staining quality), whereas mucicarmine stain was negative for intracytoplasmic mutin. Immunocytochemical studies were performed with the avidin-biotin-peroxidase technique.4 The following primary antibodies were used: keratins (CAM 5.2, monoclonal; Becton Dickinson & Co., Mountain View, Calif.), AEi/ AE3 (monoclonal; Hybritech, San Diego, Calif.), S-100 protein (polyclonal; Dako Corp., Santa Barbara, Calif.); vimentin (monoclonal; Dako), desmin (monoclonal; Amersham International Ltd., Little Chalfont, U.K.), and carcinoembryonic antigen (polyclonal and monoclonal; Dako). The results of this study are summarized in Table I. Tumor cells reacted for keratin, S-100 protein, and polyclonal carcinoembryonic antigen. A diagnosis of papillary adenocarcinoma, possibly of salivary gland origin, was made. Preoperative evaluation (including complete physical examination, chest x-ray fiim,
Volume Number
Low-grade
73 5
Table II. Reported cases of LGPA
Reported duration M/65
adenocarcinoma
593
of minor salivary glands
Author EdwardsI
papillary
Hard
palate
Follow-up
Treatment
-
Excision
No recurrence
after
1 yr Calhoun et aL6 Cady and Huttert3 Brooks et a1.5 Case 1 Case 2 Whittaker and Turner’ Case 5
Case 6
Mills
et al.?
F/51
Upper
M/48
Palate alveolar ridge
Excision
M/31
Hard
Excision
M/63
Soft palate
6 yr
Excision
F/53
Hard and soft palate
3 yr
Excision
M/56
Buccal
6 mo
Excision, lymph node dissection
Shteyer and FundoianuDayart’ Slootweg and Muller”
Recurrence
after
43
yr
palate
No recurrence 22 mo
after
F/48
Hard
palate
Several
M/50
Hard
palate
8-10 yr
Excision
M/30
Hard and soft palate Hard and soft palate Hard and soft palate Sublingual submandibular lymph node Right midface
3-4 yr
Wide excision
20 yr
Wide
No recurrence
M/42 et a1.t4
10 days (?)
Recurrence after 6 yr; reexcision; 2nd recurrence 1 yr later; radiation and reexcision (5000 rad) Recurrence after 8 yr; reexcision and radiation Recurrence after 19 yr followed by cervical lymph node metastasis Recurrence after 1 yr with cervical lymph node metastasis No recurrence 10 yr
F/71
Cracker
lip
M/5
M/71 M/76
mucosa
Soft palate
mo
Excision
excision
9 mo
Wide
3 mo (?)
Hemiglossectomy and modified neck dissection Radiation
1 mo (?) 1 wk-I
yr
excision
Resection
after
9 Yr Cervical metastasis after 7 yr No recurrence after
2 Yr No recurrence
after
3 yr No recurrence
after
2 Yr
TX, Treatment
M/74
Hard
palate
1 wk-7
yr
Excision
M/56
Hard
palate
1 wk-7
yr
Recurrence followed by electrocautery
M/52
Hard palate, submandibular
1 wk-7
yr
Resection and excision of lymph node
No recurrence after 14 yr Recurrence after 18 yr; reexcision; 2nd recurrence after 1 yr; reexcision Recurrent lymph node metastasis in neck, 3 recurrences at 1, 5, and 11 yr; final TX was neck dissection 12 yr after 1st TX; metastasis in lung and vertebra; died of tumor 20 yr after initial TX
Mostoji et al.
594
ORAL
%JKG
ORAL
MED
ORAL
?ATti0~
May
Table
1992
II. Cont’d. Treatment
F/61
study
palate
1 wk-I
yr
1 wk-7
F/42
1 wk-I
yr
Resection
F/87
Soft palate
1 wk-7
yr
M/65
Hard
palate
11 yr
yr
Resection
Hard palate, cervical lymph node Hard palate
M/60
Present
Hard
Resection, neck dissection
Follow-up No recurrence
after
9 Yr No recurrence
after
2 Yr
Resection
No recurrence 25 yr No recurrence
after after
Resection
1 yr No recurrence
after
3 Yr
bonescan,completebloodcell count, andserumchemistry) wasnegativefor metastasis andorganinvolvement.The remainingtumor wasremovedby hemimaxillectomy,through a modifiedWeber-Fergusson incision.The patient tolerated the procedurewell, andthe defectwaseventuallyobtunded with a prostheticappliance.The patient is alive and free of disease3 years after surgery. DISCUSSION According to the World Health Organization salivary gland tumor classification, tumors with a papillary pattern fall into the group of adenocarcinoma. More than 75% of the papillary, mutinous, and other unusual variants of adenocarcinoma arise in minor salivary glands. Allen et a1.sreviewed 67 casesof primary tumors of the palate. Only two tumors were papillary adenocarcinomas, which were designated LGPA. LGPA of salivary gland origin is a rare tumor with high predilection for the oral cavity in genera! and the palate in particular. This tumor has been reported in the literature under a variety of names, including papillary cystadenoma, papillary cystadenocarcinoma, and papillary-cystic adenocarcinoma.‘, 5-10An exhaustive English-language literature review revealed only 22 well-documented cases1-3T 5-14Nine casesin a seriesof 492 casesof minor salivary gland tumors reviewed by Spiro et a1.7were papillary adenocarcinomas. They labeled these tumors papillarycystic adenocarcinomas. Evans and Batsakis,15however, argued that the casesreported by Allen et a1.3 and Spiro et ah7 were polymorphous low-grade adenocarcinomas. Slootweg and Muller”’ recently reviewed 15 cases of low-grade adenocarcinomas of the oral cavity. Seven of these caseshad features similar to terminal duct adenocarcinoma, a subtype of polymorphous low-grade adenocarcinoma, and eight were LGPA.
They concluded that terminal duct adenocarcinoma and LGPA show sufficient differences, both histologically and clinically, to be considered as separate entities. Because of its rarity and its deceptively bland histologic appearance, LGPA isoften misdiagnosed.The only way to distinguish LGPA from other minor salivary gland neoplasmsin this region is by meansof a careful histopathologic examination. A major feature of the LGPA is the presence of papillary proliferations that project into cystlike spaces.The tumor cells are either cuboidal or columnar and are arranged in papillary configurations that vary from simple to complex.2 Occasional mitotic figures and moderate nuclear pleomorphism are present. Several benign and malignant lesions should be considered in the differential diagnosis.These lesions include mucopapillary cyst,16 adenomatous papillary hyperplasia, papillary cystadenoma, benign mixed tumor, adenocystic carcinoma, mucoepidermoid carcinoma, acinic cell adenocarcinoma, and polymorphous low-grade adenocarcinoma. Benign salivary tumors in this region (papillary cystadenoma in particular) could readily be differentiated from LGPA. LGPA is an unencapsulated and infiltrative tumor that exhibits somecellular pleomorphism. Other mahgnant salivary lesions, however, have characteristic histopathologic features that distinguish them from LGPA. Clinically, LGPA is a slow-growing neoplasm(Table 11). The agesof the patients in the reported cases ranged from 5 to 87 years (mean 54.7 years). Of these reported cases,15 patients were male and 7 were female. In all the reported casesLGPA originated in the minor salivary glands, predominantly in the palate (posterior; either hard or soft). The size of most tumors at the time of clinical evaluation varied from 1.5 to 3 cm. The tumors were usually covered by in-
Volume Number
Low-grade papillary adenocarcinoma 595
73 5
tact mucosa. Swelling was the most common clinical presentation, whereas occasionally ulceration and pain were additional clinical symptoms. Treatment of choice for LGPA is wide local excision, including soft tissue and bone.17 There is a tendency for local recurrence if the tumor has not been excised completely. Six of 22 reported tumors (27%) recurred locally. Local recurrence in these cases appeared to be unrelated to the duration of sym!ptoms and size of the tumor. Disease-free intervals between local recurrences in these cases varied from 12 months to 19 years. In comparison with other types of low-grade adenocarcinoma of minor salivary glands in this region, LGPA appears to have a greater tendency to metastasize. In four (17%) of the reported cases cervical lymph node metastases were noted at the time of presentation. The present case represents a classic examlple of LGPA both clinically and histopathologically. Because of the radiographic report of possible bone involvement and the patient’s age, hemimaxillectomy was considered the tl:eatment of choice by the members of the tumor board. REFERENCES 1. Whittaker JS, Turner EP. Papillary tumors of the minor salivary glands. J Clin Path01 1976;29:‘795-805. 2. Mills SE, Garland TA, Allen MS. Low-grade papillary adenocarcinoma of the palatal salivary gland origin. Am J Surg Path01 1984;8:367-74. 3. Allen MS, Fitz-Hugh GS, Marsh WL. Low-grade papillary adenocarcinoma of the palate. Cancer 1974;33:153-8. 4. Hsu SM, Raine L, Fanger H. Use of avidin-biotin-peroxidase complex (ABC) in immunoperoxidase techniques: a compar-
ison between ABC and unlabelled antibody (PAP) procedures. J Histochem Cytochem 1981;29:577-80. 5. Brooks HW, Hiebert AE, Pullman NK, Stofer BE. Papillary cystadenoma of the palate: a review of the literature and report of two new cases. ORAL SURC ORAL MED ORAL PATHOL
1956;9:1047-50.
6. Calhoun NR, Cerine FC, Mathews MJ. Papillary cystadenoma of the upper lip; report of a case. ORAL SURGORAL MED
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