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Chronic sclerosing sialadenitis or Küttner's tumor associated with a giant sialolith: a case report
Vol. 115 No. 4 April 2013
Chronic sclerosing sialadenitis or Küttner’s tumor associated with a giant sialolith: a case report Cyril Pandarakalam, BDS, MDS,a William M. Goebel, DDS, MSD,b and Bradley Seyer, DDS, MSa Southern Illinois University, Alton, Illinois
Chronic sclerosing sialadenitis (Küttner’s tumor [KT]) is a chronic inflammatory salivary gland disease primarily affecting the submandibular gland. Sialolithiasis is considered the most common etiologic factor for KT. We report a case of KT associated with a giant sialolith of the submandibular gland and a secondary smaller sialolith in the associated submandibular duct. (Oral Surg Oral Med Oral Pathol Oral Radiol 2013;115:e38-e40)
Chronic sclerosing sialadenitis was first described by Küttner in 1896 and thus is commonly referred to as Küttner’s tumor (KT).1 Despite the name, KT is not a true neoplasm, but an inflammatory disease of the salivary glands that is often difficult to distinguish clinically from a neoplasm. KT is grouped under tumor-like lesions in the World Health Organization classification of salivary gland tumors.2,3 KT is most commonly associated with the submandibular salivary gland. Clinically, KT is characterized by firm swelling of the affected gland with or without pain. Sialoliths are more often observed with KT and are considered the most common etiologic factor. Other proposed etiologic factors responsible for KT are secretory disorders, infectious agents, and immunologic responses.4-7 Recently, a relationship between autoimmunity and KT has been postulated. Recent studies showed that the pathogenesis of KT may be associated with immune-mediated processes such as immunoglobulin (Ig)-G4-related disease, which is characterized by high serum IgG4 level and tissue infiltration (including salivary gland) of IgG4-positive plasma cells.8 The distinguishing histologic features of KT are periductal sclerosis, lymphocytic infiltration, lymphocytic follicles, atrophic acini, and fibrosis. There are 4 different histologic stages depending on the severity and progression of the glandular destruction and fibrosis.9 In this report, we describe a case of KT associated A poster of this case was presented during the AAOM annual meeting held in Charleston, South Carolina, 2012. a Assistant Professor, Section of Diagnostic Sciences, Department of Applied Dental Medicine, School of Dental Medicine, Southern Illinois University, Alton, Illinois. b Professor Emeritus, Section of Diagnostic Sciences, Department of Applied Dental Medicine, School of Dental Medicine, Southern Illinois University, Alton, Illinois. Received for publication July 31, 2012; returned for revision Oct 16, 2012; accepted for publication Oct 19, 2012. © 2013 Elsevier Inc. All rights reserved. 2212-4403/$ - see front matter http://dx.doi.org/10.1016/j.oooo.2012.10.011
e38
with a giant sialolith in the submandibular gland and a second sialolith in Wharton’s duct.
CASE REPORT A 68-year-old man appeared in our outpatient dental emergency department with pain and swelling of the right submandibular region. The patient had noticed swelling in the region about 4 years earlier, but did not seek professional medical attention at that time. The patient reported that the lesion had gradually increased in size and had recently become painful. His medical history was unremarkable except for hypertension and dyslipidemia, managed with medications. He has no drug allergies and smokes a pack of cigarettes a day. Visual inspection did not reveal the swelling extraorally because of the patient’s thick, full facial hair (Figure 1). Extraoral examination revealed that the swelling was localized to the right submandibular region with a hard consistency and was tender on palpation. Intraoral examination revealed induration with a slight elevation of the right floor of the mouth, with absent salivary flow from the right Wharton’s duct. A hard, white material was noted at the orifice of Wharton’s duct (Figure 2). Occlusal (Figure 3) and panoramic (Figure 4) radiographs revealed 2 separate radiopaque masses of the right submandibular gland and the duct. Computed tomographic scan was obtained (Figure 5) and impression was a large calcification in the right submandibular gland resulting in enlargement and some edema of the gland. Fine-needle aspiration cytology results were inconclusive. The patient was referred to an otolaryngologist, who performed an excision of the right submandibular salivary gland along with removal of an impacted sialolith measuring approximately 4.0 ⫻ 2.5 cm. A second smaller sialolith measuring 8 ⫻ 3 mm was also removed from the anterior aspect of the right Wharton’s duct. The histopathologic diagnosis of the soft-tissue specimen was KT (Figure 6).
DISCUSSION KT is an underreported condition of the salivary gland that mainly affects the submandibular salivary gland, although parotid gland, sublingual gland, and minor salivary gland involvement have been reported.10,11,12,13 In
OOOO Volume 115, Number 4
Fig. 1. The patient’s thick facial hair partially obscured the extraoral swelling of the right side.
Fig. 2. Intraoral exam revealed a white, calcified hard material at the Wharton’s duct orifice.
CASE REPORT Pandarakalam, Goebel and Seyer e39
Fig. 4. Panoramic radiograph revealing a large, lobulated, radiopaque mass in the right submandibular region corresponding to the sialolith.
Fig. 5. Computed tomography scan of the right submandibular region demonstrating the sialolith.
Fig. 3. Occlusal radiograph showing an elongated radiopaque mass in the right floor of the mouth corresponding to the smaller ductal sialolith.
this case, the patient noticed the swelling 4 years earlier, with gradual increase in size, but only with recent development of pain. Sialoliths are considered the most common etiologic factor associated with KT and are found in about 50% to 80% of cases, the majority of which are extraglandular.9 However, whether the sia-
Fig. 6. Histopathologic findings revealed chronic inflammatory infiltrate associated with acinar atrophy and periductal sclerosis (hematoxylin– eosin stain, magnification ⫻10).
lolith is the cause or the result of the inflammatory process in the involved gland is not well understood. Our case involved the presence of a smaller ductal
ORAL AND MAXILLOFACIAL PATHOLOGY e40 Pandarakalam, Goebel and Seyer
stone and a giant stone in the gland. Given the information available to us, we cannot speculate as to which occurred first. As mentioned previously, immune-mediated processes are also suspected in the pathogenesis of KT. Immunohistochemical tissue analysis and serum IgG quantification were not performed in this case because follow-up protocols for sialadenitis have not been formalized. As universally accepted clinical follow-up protocols are developed, immunohistochemistry might become routine. Sialoliths, also referred to as salivary calculi or salivary gland stones, are most commonly seen in the submandibular gland, followed by parotid and rarely sublingual and minor salivary glands. The majority of the submandibular sialoliths are found in Wharton’s duct because of its tortuous path and more viscous saliva. Pain and swelling may occur during mealtime, the severity of which varies depending on the degree of obstruction and pressure from stimulated salivary flow. Sialoliths involving multiple glands and multiple sialoliths in the same gland are extremely rare. Sialoliths usually measure about 1 to 10 mm, but there are larger sialoliths measuring more than 1 cm. These are referred to as “sialoliths of unusual size” and sialoliths measuring more than 3.5 cm are referred to as “giant sialoliths.”14,15 In our case, a giant sialolith (4.0 ⫻ 2.5 cm) was found in the submandibular gland. Differential diagnosis of glandular swelling seen in KT includes a benign lymphoepithelial lesion and granulomatous or radiation-induced sialadenitis. The clinical diagnosis of KT is further complicated because some patients present with only asymptomatic hard swelling that may resemble a salivary gland tumor. Fine-needle aspiration cytology may help to rule out malignancy. Sialoliths, depending on the degree of calcification and size, can be evaluated using panoramic radiographs and occlusal radiographs. Giant sialoliths are usually radiopaque and are easily seen on plane imaging. Computed tomographic scans can be used to evaluate the size and extension of the growth of the sialolith.11,15 Histologic diagnosis of KT is made by the presence of lymphocytic inflammation, periductal sclerosis, acinar atrophy, and fibrosis. There are 4 histologic stages based on the progression and severity. Stage 1 exhibits focal chronic inflammatory aggregates around the salivary ducts containing inspissated secretion. In stage 2, there is marked diffuse lymphocytic infiltration and more severe periductal fibrosis. Reduction of secretory gland parenchyma, secondary lymphoid follicle formation with reactive germinal centers, and extensive fibrosis are seen in Stage 3, finally progressing in Stage 4 to destruction of the gland with marked parenchymal loss and sclerosis.6 The treatment of choice for KT is surgical excision of the involved gland.
OOOO April 2013
KT is an underrecognized, underreported condition. We believe that this is the first report of occurrence of KT associated with multiple sialoliths in the same gland: a giant sialolith involving the submandibular gland and a secondary smaller sialolith in the associated submandibular duct. REFERENCES ¨ ber entzündliche tumoren der submaxillarspeichel1. Küttner H. U drüse. Bruns‘Beitr Klin Chir 1896;15:815-28. 2. Seifert G. Tumour-like lesions of the salivary glands. The new WHO classification. Pathol Res Pract 1992;188:836-46. 3. Seifert G, Sobin LH. The World Health Organization’s histological classification of salivary gland tumors. A commentary on the second edition. Cancer 1992;70:379-85. 4. Harrison JD, Epivatianos A, Bhatia SN. Role of microliths in the aetiology of chronic submandibular sialadenitis: a clinicopathological investigation of 154 cases. Histopathology 1997;31:237-51. 5. Chan JK. Kuttner tumor (chronic sclerosing sialadenitis) of the submandibular gland: an underrecognized entity. Adv Anat Pathol 1998;5:239-51. 6. Seifert G, Donath K. [On the pathogenesis of the Kuttner tumor of the submandibular gland—analysis of 349 cases with chronic sialadenitis of the submandibular (author’s transl)]. HNO 1977;25:81-92. 7. Tiemann M, Teymoortash A, Schrader C, Werner JA, Parwaresch R, Seifert G, Klöppel G. Chronic sclerosing sialadenitis of the submandibular gland is mainly due to a T lymphocyte immune reaction. Mod Pathol 2002;15:845-52. 8. Abe T, Sato T, Tomaru Y, Sakata Y, Kokabu S, Hori N, et al. Immunoglobulin G4-related sclerosing sialadenitis: report of two cases and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2009;108:544-50. Review. 9. Ellis GL, Auclair PL. Chronic sclerosing sialadenitis. Atlas of tumour pathology, 3rd series, fasc 10. Washington, DC: Armed Forces Institute of Pathology; 1996. pp. 419 –21. 10. Blanco M, Mesko T, Cura M, Cabello-Inchausti B. Chronic sclerosing sialadenitis (Kuttner’s tumor): unusual presentation with bilateral involvement of major and minor salivary glands. Ann Diagn Pathol 2003;7:25-30. 11. Beriat GK, Akmansu SH, Kocatürk S, Ataog˘lu O. Chronic sclerosing sialadenitis (Küttner’s tumour) of the parotid gland. Malays J Med Sci 2010;17:57-61. 12. de Vicente JC, López-Arranz E, García J, López-Arranz JS. Chronic sclerosing sialadenitis of the parotid gland. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2003;96:77-80. 13. Williams HK, Connor R, Edmondson H. Chronic sclerosing sialadenitis of the submandibular and parotid glands: a report of a case and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2000;89:720-3. 14. Siddiqui SJ. Sialolithiasis: an unusually large submandibular salivary stone. Br Dent J 2002;193:89-91. 15. Rai M, Burman R. Giant submandibular sialolith of remarkable size in the comma area of Wharton’s duct: a case report. J Oral Maxillofac Surg 2009;67:1329-32. Reprint requests: Cyril Pandarakalam, BDS, MDS Assistant Professor, Section of Diagnostic Sciences Department of Applied Dental Medicine, School of Dental Medicine Southern Illinois University 2800 College Avenue Alton, Illinois 62002. [email protected]
Chronic sclerosing sialadenitis or Küttner’s tumor associated with a giant sialolith: a case report Cyril Pandarakalam, BDS, MDS,a William M. Goebel, DDS, MSD,b and Bradley Seyer, DDS, MSa Southern Illinois University, Alton, Illinois
Chronic sclerosing sialadenitis (Küttner’s tumor [KT]) is a chronic inflammatory salivary gland disease primarily affecting the submandibular gland. Sialolithiasis is considered the most common etiologic factor for KT. We report a case of KT associated with a giant sialolith of the submandibular gland and a secondary smaller sialolith in the associated submandibular duct. (Oral Surg Oral Med Oral Pathol Oral Radiol 2013;115:e38-e40)
Chronic sclerosing sialadenitis was first described by Küttner in 1896 and thus is commonly referred to as Küttner’s tumor (KT).1 Despite the name, KT is not a true neoplasm, but an inflammatory disease of the salivary glands that is often difficult to distinguish clinically from a neoplasm. KT is grouped under tumor-like lesions in the World Health Organization classification of salivary gland tumors.2,3 KT is most commonly associated with the submandibular salivary gland. Clinically, KT is characterized by firm swelling of the affected gland with or without pain. Sialoliths are more often observed with KT and are considered the most common etiologic factor. Other proposed etiologic factors responsible for KT are secretory disorders, infectious agents, and immunologic responses.4-7 Recently, a relationship between autoimmunity and KT has been postulated. Recent studies showed that the pathogenesis of KT may be associated with immune-mediated processes such as immunoglobulin (Ig)-G4-related disease, which is characterized by high serum IgG4 level and tissue infiltration (including salivary gland) of IgG4-positive plasma cells.8 The distinguishing histologic features of KT are periductal sclerosis, lymphocytic infiltration, lymphocytic follicles, atrophic acini, and fibrosis. There are 4 different histologic stages depending on the severity and progression of the glandular destruction and fibrosis.9 In this report, we describe a case of KT associated A poster of this case was presented during the AAOM annual meeting held in Charleston, South Carolina, 2012. a Assistant Professor, Section of Diagnostic Sciences, Department of Applied Dental Medicine, School of Dental Medicine, Southern Illinois University, Alton, Illinois. b Professor Emeritus, Section of Diagnostic Sciences, Department of Applied Dental Medicine, School of Dental Medicine, Southern Illinois University, Alton, Illinois. Received for publication July 31, 2012; returned for revision Oct 16, 2012; accepted for publication Oct 19, 2012. © 2013 Elsevier Inc. All rights reserved. 2212-4403/$ - see front matter http://dx.doi.org/10.1016/j.oooo.2012.10.011
e38
with a giant sialolith in the submandibular gland and a second sialolith in Wharton’s duct.
CASE REPORT A 68-year-old man appeared in our outpatient dental emergency department with pain and swelling of the right submandibular region. The patient had noticed swelling in the region about 4 years earlier, but did not seek professional medical attention at that time. The patient reported that the lesion had gradually increased in size and had recently become painful. His medical history was unremarkable except for hypertension and dyslipidemia, managed with medications. He has no drug allergies and smokes a pack of cigarettes a day. Visual inspection did not reveal the swelling extraorally because of the patient’s thick, full facial hair (Figure 1). Extraoral examination revealed that the swelling was localized to the right submandibular region with a hard consistency and was tender on palpation. Intraoral examination revealed induration with a slight elevation of the right floor of the mouth, with absent salivary flow from the right Wharton’s duct. A hard, white material was noted at the orifice of Wharton’s duct (Figure 2). Occlusal (Figure 3) and panoramic (Figure 4) radiographs revealed 2 separate radiopaque masses of the right submandibular gland and the duct. Computed tomographic scan was obtained (Figure 5) and impression was a large calcification in the right submandibular gland resulting in enlargement and some edema of the gland. Fine-needle aspiration cytology results were inconclusive. The patient was referred to an otolaryngologist, who performed an excision of the right submandibular salivary gland along with removal of an impacted sialolith measuring approximately 4.0 ⫻ 2.5 cm. A second smaller sialolith measuring 8 ⫻ 3 mm was also removed from the anterior aspect of the right Wharton’s duct. The histopathologic diagnosis of the soft-tissue specimen was KT (Figure 6).
DISCUSSION KT is an underreported condition of the salivary gland that mainly affects the submandibular salivary gland, although parotid gland, sublingual gland, and minor salivary gland involvement have been reported.10,11,12,13 In
OOOO Volume 115, Number 4
Fig. 1. The patient’s thick facial hair partially obscured the extraoral swelling of the right side.
Fig. 2. Intraoral exam revealed a white, calcified hard material at the Wharton’s duct orifice.
CASE REPORT Pandarakalam, Goebel and Seyer e39
Fig. 4. Panoramic radiograph revealing a large, lobulated, radiopaque mass in the right submandibular region corresponding to the sialolith.
Fig. 5. Computed tomography scan of the right submandibular region demonstrating the sialolith.
Fig. 3. Occlusal radiograph showing an elongated radiopaque mass in the right floor of the mouth corresponding to the smaller ductal sialolith.
this case, the patient noticed the swelling 4 years earlier, with gradual increase in size, but only with recent development of pain. Sialoliths are considered the most common etiologic factor associated with KT and are found in about 50% to 80% of cases, the majority of which are extraglandular.9 However, whether the sia-
Fig. 6. Histopathologic findings revealed chronic inflammatory infiltrate associated with acinar atrophy and periductal sclerosis (hematoxylin– eosin stain, magnification ⫻10).
lolith is the cause or the result of the inflammatory process in the involved gland is not well understood. Our case involved the presence of a smaller ductal
ORAL AND MAXILLOFACIAL PATHOLOGY e40 Pandarakalam, Goebel and Seyer
stone and a giant stone in the gland. Given the information available to us, we cannot speculate as to which occurred first. As mentioned previously, immune-mediated processes are also suspected in the pathogenesis of KT. Immunohistochemical tissue analysis and serum IgG quantification were not performed in this case because follow-up protocols for sialadenitis have not been formalized. As universally accepted clinical follow-up protocols are developed, immunohistochemistry might become routine. Sialoliths, also referred to as salivary calculi or salivary gland stones, are most commonly seen in the submandibular gland, followed by parotid and rarely sublingual and minor salivary glands. The majority of the submandibular sialoliths are found in Wharton’s duct because of its tortuous path and more viscous saliva. Pain and swelling may occur during mealtime, the severity of which varies depending on the degree of obstruction and pressure from stimulated salivary flow. Sialoliths involving multiple glands and multiple sialoliths in the same gland are extremely rare. Sialoliths usually measure about 1 to 10 mm, but there are larger sialoliths measuring more than 1 cm. These are referred to as “sialoliths of unusual size” and sialoliths measuring more than 3.5 cm are referred to as “giant sialoliths.”14,15 In our case, a giant sialolith (4.0 ⫻ 2.5 cm) was found in the submandibular gland. Differential diagnosis of glandular swelling seen in KT includes a benign lymphoepithelial lesion and granulomatous or radiation-induced sialadenitis. The clinical diagnosis of KT is further complicated because some patients present with only asymptomatic hard swelling that may resemble a salivary gland tumor. Fine-needle aspiration cytology may help to rule out malignancy. Sialoliths, depending on the degree of calcification and size, can be evaluated using panoramic radiographs and occlusal radiographs. Giant sialoliths are usually radiopaque and are easily seen on plane imaging. Computed tomographic scans can be used to evaluate the size and extension of the growth of the sialolith.11,15 Histologic diagnosis of KT is made by the presence of lymphocytic inflammation, periductal sclerosis, acinar atrophy, and fibrosis. There are 4 histologic stages based on the progression and severity. Stage 1 exhibits focal chronic inflammatory aggregates around the salivary ducts containing inspissated secretion. In stage 2, there is marked diffuse lymphocytic infiltration and more severe periductal fibrosis. Reduction of secretory gland parenchyma, secondary lymphoid follicle formation with reactive germinal centers, and extensive fibrosis are seen in Stage 3, finally progressing in Stage 4 to destruction of the gland with marked parenchymal loss and sclerosis.6 The treatment of choice for KT is surgical excision of the involved gland.
OOOO April 2013
KT is an underrecognized, underreported condition. We believe that this is the first report of occurrence of KT associated with multiple sialoliths in the same gland: a giant sialolith involving the submandibular gland and a secondary smaller sialolith in the associated submandibular duct. REFERENCES ¨ ber entzündliche tumoren der submaxillarspeichel1. Küttner H. U drüse. Bruns‘Beitr Klin Chir 1896;15:815-28. 2. Seifert G. Tumour-like lesions of the salivary glands. The new WHO classification. Pathol Res Pract 1992;188:836-46. 3. Seifert G, Sobin LH. The World Health Organization’s histological classification of salivary gland tumors. A commentary on the second edition. Cancer 1992;70:379-85. 4. Harrison JD, Epivatianos A, Bhatia SN. Role of microliths in the aetiology of chronic submandibular sialadenitis: a clinicopathological investigation of 154 cases. Histopathology 1997;31:237-51. 5. Chan JK. Kuttner tumor (chronic sclerosing sialadenitis) of the submandibular gland: an underrecognized entity. Adv Anat Pathol 1998;5:239-51. 6. Seifert G, Donath K. [On the pathogenesis of the Kuttner tumor of the submandibular gland—analysis of 349 cases with chronic sialadenitis of the submandibular (author’s transl)]. HNO 1977;25:81-92. 7. Tiemann M, Teymoortash A, Schrader C, Werner JA, Parwaresch R, Seifert G, Klöppel G. Chronic sclerosing sialadenitis of the submandibular gland is mainly due to a T lymphocyte immune reaction. Mod Pathol 2002;15:845-52. 8. Abe T, Sato T, Tomaru Y, Sakata Y, Kokabu S, Hori N, et al. Immunoglobulin G4-related sclerosing sialadenitis: report of two cases and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2009;108:544-50. Review. 9. Ellis GL, Auclair PL. Chronic sclerosing sialadenitis. Atlas of tumour pathology, 3rd series, fasc 10. Washington, DC: Armed Forces Institute of Pathology; 1996. pp. 419 –21. 10. Blanco M, Mesko T, Cura M, Cabello-Inchausti B. Chronic sclerosing sialadenitis (Kuttner’s tumor): unusual presentation with bilateral involvement of major and minor salivary glands. Ann Diagn Pathol 2003;7:25-30. 11. Beriat GK, Akmansu SH, Kocatürk S, Ataog˘lu O. Chronic sclerosing sialadenitis (Küttner’s tumour) of the parotid gland. Malays J Med Sci 2010;17:57-61. 12. de Vicente JC, López-Arranz E, García J, López-Arranz JS. Chronic sclerosing sialadenitis of the parotid gland. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2003;96:77-80. 13. Williams HK, Connor R, Edmondson H. Chronic sclerosing sialadenitis of the submandibular and parotid glands: a report of a case and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2000;89:720-3. 14. Siddiqui SJ. Sialolithiasis: an unusually large submandibular salivary stone. Br Dent J 2002;193:89-91. 15. Rai M, Burman R. Giant submandibular sialolith of remarkable size in the comma area of Wharton’s duct: a case report. J Oral Maxillofac Surg 2009;67:1329-32. Reprint requests: Cyril Pandarakalam, BDS, MDS Assistant Professor, Section of Diagnostic Sciences Department of Applied Dental Medicine, School of Dental Medicine Southern Illinois University 2800 College Avenue Alton, Illinois 62002. [email protected]