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Primary mucoepidermoid carcinoma of Stensen's duct
81
HAAR ET AL
J Oral Maxillofac
Surg
49~81-84, 1991
Primary Mucoepidermoid Carcinoma Stensen’s Duct
of
JEAN G. HAAR, DDS, MD,* JAMES WOYTASH, DDS, MS, MD,t AND JOHN E. ASIRWATHAM, MD*
Primary carcinomas arising from Stensen’s duct (PCSD) are uncommon, with fewer than 20 cases reported in the English literature.‘-13 An additional case of PCSD complicated by a second concurrent primary tumor in the opposite parotid gland is presented. Multiple tumors of the salivary glands following a history of radiation treatment to the head and neck for a variety of benign conditions have recently been reported. 16*17Our case is unique in that the patient developed a primary mucoepidermoid carcinoma (MEC) of the left Stensen’s duct followed by a MEC of the right parotid gland 35 to 42 years after receiving radiation therapy for facial acne. To the best of our knowledge, no such case has been documented previously in the English literature. A review of the published reports of PCSD and the role of previous radiation therapy will be discussed.
duct (Figs 1 and 2). The left parotid gland showed atrophic parenchymal changes. One month later, the patient presented with minimal swelling of the right buccal mucosa. A sialogram was performed and interpreted as abnormal, with nodular beading and poorly defined ducts. Because of persistence of
Report of a Case
For the purpose of clarity, the following discussion will be separated into three salient sections: first, PCSD; second, multiple salivary gland tu-
A 57-year-old white man was first seen in July 1984for recurrent right parotid gland swelling of 6 months duration. His past medical history included radiation treatment for acne at ages 15 and 20. No clinical abnormalities were present in the right parotid gland region, but a l-cm mass was noted adjacent to the left Stensen’s duct. Plain radiographs of the left parotid gland were negative for calculi. No facial weakness was noted. In September 1984, due to progressive enlargement of the nodule, a left parotid lobectomy and excision of Stensen’s duct was done. This revealed a circumscribed, l-cm nodular mass attached to the wall of Stensen’s duct. The histologic report, following use of mucicarmine stains, indicated high-grade intraductal MEC of Stensen’s
Received from Buffalo General Hospital, Buffalo, NY. * Chief, Department of Otolaryngology/Head and Neck Surgery. I’ Fellow, Department of Surgical Pathology. $ Associate Pathologist, Department of Surgical Pathology. Address correspondence and reprint requests to Dr Woytash: Department of Surgical Pathology, Buffalo General Hospital, 100 High St, Buffalo, NY 14203. 0 1991 American geons
Association
0278-2391191/4901-0014$3.00/O
of Oral and Maxillofacial
Sur-
the swelling, surgical exploration followed by a right parotid lobectomy was done in November 1984. The histologic report described dysplastic changes consisting of atypical squamous epithelium in the interlobular ducts (Fig 3). Two years later, in July 1986, due to the appearance of two nodules in the right buccal mucosa, a total residual parotidectomy was done. The histologic report was highgrade MEC involving the glandular parenchyma (Fig 4). Postoperatively, the patient received 5,600 rad of external irradiation to the right cheek. At the time of this report, 6 years postoperatively, there is neither local recurrence nor distant metastases.
Discussion
mors; and third, the role of previous head and neck radiation related to development of neoplasms. Table 1 is a compilation of PCSD cases reported in the English literature. Goforth’ in 1927 apparently documented one of the first cases, although others disputed the diagnosis.3*8 If one is to adopt a purist’s view of such tumors, then only those histologically arising from Stensen’s duct are permitted 1.11.12 The clinical presentation in our case of a .painless nodule located near the orifice of Stensen’s duct is in keeping with the reported cases. Further, in our case, that a zone of transition from the columnar epithelium of Stensen’s duct to the carcinomatous region was identified and that the gland proper was not involved are consistent with these types of tumors. These findings fulfill the criteria previously set forth that PCSD should be distinct from both the gland parenchyma and the buccal mucosa and, histologically, the lesion should be an epidermoid, mucoepidermoid, or undifferentiated cancer.‘.‘2 This differential diagnosis of squamous cell carcinoma,
82
FIGURE 1. Left Stensen’s duct with high-grade intraluminal MEC (hematoxylin-eosin, original magnification ~6.6).
CARCINOMA OF STENSEN’S
DUCT
FIGURE 3. Interlobular duct of right parotid gland with zone of transition to an area of moderate epithelial dysplasia (hematoxylin-eosin, original magnification X33).
MEC, and undifferentiated carcinoma is in keeping with the theories of both Eversole and Batsakis regarding salivary gland histogenesis.25,26 By virtue of their rarity, the treatment of PCSD must be established on an individual basis with general principles set forth by Owens et al as well as Clairmont.9,10 These authors advocate surgical excision with an extraoral approach, as done in our case, to obtain adequate exposure, adequate margins, and identification of the facial nerve. Frechette et al set forth criteria of treatment based on clinical staging and advocate radiation therapy as an adjunctive procedure.‘* In our case, consideration was given to postoperative radiotherapy to the left cheek. However, due to the fact that the carcinoma was localized to the duct with no involvement of the parotid gland, we elected not to deliver postoperative radiation. The second striking aspect in our case was the development of in situ neoplasia in the right parotid gland (Fig 3) followed by MEC of the residual right parotid gland (Fig 4). Trumbull and Frazell reported
that 1% of patients with salivary gland tumors have involvement of more than one gland or multiple foci in the same gland.21 A more recent study by Katz and Preston-Martin reported that 13% of their patients had multiple salivary gland tumors; these patients had a history of previous irradiation to the head and neck, as did our case.17 The concept of head and neck neoplasms arising in areas previously irradiated is not novel. Duffy and Fitzgerald first described the relationship between thymic irradiation and development of thyroid carcinoma.24 Rice et al in 1976 cited 23 cases of malignant salivary gland tumors associated with postirradiation and also reported one additional case following radiation treatment for acne.*’ These authors state that MEC is the most frequent lesion to develop and also that a latent period of 20 years or more is required for neoplastic development. Schneider et al in 1986 reviewed 2,561 patients given external radiation before 15 years of age and found that 65 of these patients had developed sali-
FIGURE 2. Higher-power view of Figure 1 showing transition from ductal epithelium to a high-grade MEC (hematoxylin-eosin, original magnification X33).
FIGURE 4. Right parotid gland with high-grade MEC similar to the tumor that arose in the left Stensen’s duct (hematoxylineosin, original magnification x 132).
83
HAAR ET AL
Table 1.
Primary Carcinoma of Stensen’s Duct Location
Author(s)
Age
Sex
Goforth (1927)
60
F
Left distal duct
Lyall & Golcomb (1954) Peracchio (1958)
40
M
51
Maisel et al (1959)
Diagnosis
Treatment
Outcome
Local excision and radium
Died 1% yr later with clinical metastases
Left posterior to orifice
Anaplastic squamous cell carcinoma Squamous cell carcinoma
7,900 rad after biopsy
Free of disease 18 mo follow-up
F
Left, 1.5 cm from orifice
Squamous cell carcinoma
Free of disease 4 mo after therapy
53
M
Right, 1 cm posterior to orifice
Squamous cell carcinoma
Gaisforth et al (1965) Gaisforth et al (1965) Gaisforth et al (1965) Vigorita et al (1980) Owens et al (1982)
55
F
Left duct
Mucoepidermoid carcinoma
2,828 rad plus 12 (2 mg) radon needles Radical excision followed by dissection and radical excision of maxillary bone and soft palate Parotidectomy and neck dissection
25
F
Right buccal
Mucoepidermoid carcinoma
No recurrence follow-up
?
M
Right duct
Mucoepidermoid carcinoma
Superficial parotidectomy and radiation Parotidectomy
82
M
Left, 1 cm from orifice
Squamous cell carcinoma
Free of disease 12 mo later
62
M
Right duct
Squamous cell carcinoma
Clairmont et al ( 1979) Frechette et al (1984) Present case
53
F
Right duct
Mucoepidermoid carcinoma
Left parotidectomy, radical neck dissection Parotidectomy and 4,540 rad to neck and 5,000 rad to primary site Parotidectomy
24
M
Left duct
Undifferentiated carcinoma
Not stated
57
M
Left near orifice
Mucoepidermoid carcinoma
Superficial parotidectomy and 6,240 rad Superficial parotidectomy
vary tumors and had an increased frequency of thyroid neoplasms. I9 Palmer et al, in their study of 662 patients with salivary gland tumors, found that 12 of the patients received radiation for acne.23 Swelstad et al documented 13 salivary gland neoplasms in 18 patients with a history of prior radiation to the head and neck; 7 of these patients received radiation for facial acne.** Previous head and neck radiation in childhood and infancy is a factor in the pathogenesis of neoplasms involving the thyroid and also the salivary glands, with a latent period ranging from 10 to 25 years.‘6-23 However, neither the duration of the risk nor the role of chronic sialadenitis in the development of salivary gland carcinoma following irradiation have been defined. References 1. Batsakis JC (ed): Tumors of the head and neck. Baltimore, MD, Williams & Wilkins, 1979, p 45
Free of disease 20 mo later
Died of recurrence in postoperative period 18 mo
Follow-up not stated
Free of disease
Not stated
No recurrence
2. Goforth JL: Carcinoma developing in the parotid (Stensen’s) duct. Am J Med Sci 173:624, 1927 3. Figi FA, Rowland WD: Primary tumors of Stensen’s and Wharton’s ducts. Arch Otolaryngol40:175, 1944 4. Lyall D, Golcomb FM: Carcinoma of Stensen’s duct. Ann Surg 139:364, 1954 5. Beyer T, Blair J, Hamilton P: Adenocarcinoma of the parotid duct. Arch Otolaryngol 63: 196, 1956 6. Peracchio RL: Primary squamous cell carcinoma of Stensen’s duct. Oral Surg 11:123, 1958 7. Maisel B, Pearce C, Connolly J, et al: Carbon-black carcinoma of Stensen’s duct. Arch Surg 78:331, 195!) 8. Gaisforth JC, Hanna D, Sotereanos G: Primary (cancer of Stensen’s duct. Arch Otolaryngol 82:45, 1965 9. Clairmont AA, Hanna D, Anderson V: Carcinoma of Stensen’s duct. Ann Plast Surg 2:158, 1979 10. Owens 0, Fligiel A, Ward P: Primary carcinoma. of Stensen’s duct. Otolaryngol 90:671, 1982 11. Vigorita V, Huvos A, Gerold F: Squamous cell carcinoma of Stensen’s duct. Head Neck Surg 2:513, 1980 12. Frechette C, Demetris A, Barnes E, et al: Primary carcinoma of Stensen’s duct. J Surg Oncol 27: 1, 1984 13. Carpenter R, Watkins R, Thomas J: Primary carcinoma of Stensen’s duct. Br J Surg 73:926, 1986 14. Wolfe S: Salivary duct carcinoma. Arch Otolarygol 101:398, 1975
84
HYPOPROTHROMBINEMIA:
15. Fayemi A, Toker C: Salivary duct carcinoma. Arch Otolaryngol 99:366, 1974 16. Schneider A, Shore-Freedman E, Weinstein RA: Radiation induced thyroid and other head and neck tumors. J Clin Endocrinol Metah 63: 107, 1986 17. Katz A, Preston-Martin S: Salivary gland tumors and previous radiotherapy to the head or neck. Am J Surg 147:345, 1984
21. 22.
23.
18. Maxon H, Saengev E, Buncher C, et al: Radiation associ-
ated carcinoma of the salivary glands. Ann Otol Rhino1 Laryngol90: 107, 1981 19. Schneider A, Favus M, Stachura M: Salivary gland neoplasms as a late consequence of head and neck irradiation. Ann Intern Med 87:160, 1977 20. Rice D, Batsakis J, McClatchey K: Post irradiation malig-
J Oral Maxillofac 49:84-87.
24. 25. 26.
REPORT OF A CASE
nant salivary gland tumor. Arch Otolaryngol 102:699, 1976 Tumbull A, Frazell E: Multiple tumors of major salivary glands. Am J Surg 118:434,-1969 Swelstad J. Scanlon E. Oviedo M. et al: Irradiation induced polyglandular neoplasia of the head and neck. Am J Surg 135:820, 1978 Palmer J, Mustard R, Simpson W: Irradiation as an etiologic fact in tumors of the thyroid, parathyroid, and salivary glands. Can J Surg 23:39, 1980 Duffy B, Fitzgerald P: Thyroid cancer in childhood and adolescence. J Clin Endocrinol 10:1296, 1950 Eversole L: Histogenic classification of salivary tumors. Arch Path01 92:433, 1971 Regezi J, Batsakis J: Histogenesis of salivary gland neoplasms. Otol Clin 10:297, 1977
Surg
1991
Hypoprothrombinemia (Factor II Deficiency): Report of a Case and Review of Literature ABDOLLAH RAHIMI, DDS,* RAWLE PHILBERT, DDS,t J. HAMIL WILLOUGHBY, DDS,$ AND DUDLEY S. JACKMAN, DMD§ Hypoprothrombinemia is a rare deficiency in the coagulation protein cascade. Only 10 cases of this inherited coagulation disorder have been reported in the literature. This report documents the management of a 45-year-old Hispanic man with known hypoprothrombinemia who presented to our service for multiple surgical odontectomies. Review of the Literature Quick, Piscotta, and Hussey reported the first case of factor II deficiency in 1955. ’ This disorder of prothrombin synthesis is categorized as hypopro-
Received from the Department of Oral and Maxillofacial Surgery, Harlem Hospital Center, New York. * Chief Resident. t Third-Year Resident. $ Chief. 5 Associate Chief. Address correspondence and reprint requests to Dr Willoughby: Department of Oral and Maxillofacial Surgery, Harlem Hospital Center, Martin Luther King Pavilion, Room 6215, 506 Lenox Ave, New York, NY 10037. 0 1991 American
Association
of Oral and Maxillofacial Sur-
geons 0278-2391/91/4901-0015$3.00/O
thrombinemia or dysprothrombinemia.’ Hypoprothrombinemia denotes a decrease in the synthesis or the absence of synthesis of a normal prothrombin polypeptide chain and a decreased level of factor II is described as the antigen. 3-5Dysprothrombinemia production of an abnormal prothrombin polypeptide chain with decreased or absent biological activity and near-normal levels of factor II antigen.3T6 Since 1970, 10 cases of hereditary hypoprothrombinemia documented by chemical or immunochemical assays have been reported.3Y4 The majority of the cases of hereditary hypoprothrombinemia have been among individuals of Mediterranean descent.3T5 The frequency of the hypoprothrombinemia gene has been estimated as 2110,000 in mainland Italian and Hispanic populations. Dysprothrombinemia is an autosomal recessive disorder. Patients with the heterozygous form have approximately 50% of normal levels of prothrombin and do not have bleeding manifestations. Bleeding manifestations have been reported with prothrombin levels ranging from 25% to less than 2%, with most reported cases in the range of 5% to 16%.2V3 In hypoprothrombinemia, the prothrombin time (PT) may or may not be prolonged and the test is not a very sensitive indicator of the amount of prothrombin available for coagulation. Levels of 1% to
HAAR ET AL
J Oral Maxillofac
Surg
49~81-84, 1991
Primary Mucoepidermoid Carcinoma Stensen’s Duct
of
JEAN G. HAAR, DDS, MD,* JAMES WOYTASH, DDS, MS, MD,t AND JOHN E. ASIRWATHAM, MD*
Primary carcinomas arising from Stensen’s duct (PCSD) are uncommon, with fewer than 20 cases reported in the English literature.‘-13 An additional case of PCSD complicated by a second concurrent primary tumor in the opposite parotid gland is presented. Multiple tumors of the salivary glands following a history of radiation treatment to the head and neck for a variety of benign conditions have recently been reported. 16*17Our case is unique in that the patient developed a primary mucoepidermoid carcinoma (MEC) of the left Stensen’s duct followed by a MEC of the right parotid gland 35 to 42 years after receiving radiation therapy for facial acne. To the best of our knowledge, no such case has been documented previously in the English literature. A review of the published reports of PCSD and the role of previous radiation therapy will be discussed.
duct (Figs 1 and 2). The left parotid gland showed atrophic parenchymal changes. One month later, the patient presented with minimal swelling of the right buccal mucosa. A sialogram was performed and interpreted as abnormal, with nodular beading and poorly defined ducts. Because of persistence of
Report of a Case
For the purpose of clarity, the following discussion will be separated into three salient sections: first, PCSD; second, multiple salivary gland tu-
A 57-year-old white man was first seen in July 1984for recurrent right parotid gland swelling of 6 months duration. His past medical history included radiation treatment for acne at ages 15 and 20. No clinical abnormalities were present in the right parotid gland region, but a l-cm mass was noted adjacent to the left Stensen’s duct. Plain radiographs of the left parotid gland were negative for calculi. No facial weakness was noted. In September 1984, due to progressive enlargement of the nodule, a left parotid lobectomy and excision of Stensen’s duct was done. This revealed a circumscribed, l-cm nodular mass attached to the wall of Stensen’s duct. The histologic report, following use of mucicarmine stains, indicated high-grade intraductal MEC of Stensen’s
Received from Buffalo General Hospital, Buffalo, NY. * Chief, Department of Otolaryngology/Head and Neck Surgery. I’ Fellow, Department of Surgical Pathology. $ Associate Pathologist, Department of Surgical Pathology. Address correspondence and reprint requests to Dr Woytash: Department of Surgical Pathology, Buffalo General Hospital, 100 High St, Buffalo, NY 14203. 0 1991 American geons
Association
0278-2391191/4901-0014$3.00/O
of Oral and Maxillofacial
Sur-
the swelling, surgical exploration followed by a right parotid lobectomy was done in November 1984. The histologic report described dysplastic changes consisting of atypical squamous epithelium in the interlobular ducts (Fig 3). Two years later, in July 1986, due to the appearance of two nodules in the right buccal mucosa, a total residual parotidectomy was done. The histologic report was highgrade MEC involving the glandular parenchyma (Fig 4). Postoperatively, the patient received 5,600 rad of external irradiation to the right cheek. At the time of this report, 6 years postoperatively, there is neither local recurrence nor distant metastases.
Discussion
mors; and third, the role of previous head and neck radiation related to development of neoplasms. Table 1 is a compilation of PCSD cases reported in the English literature. Goforth’ in 1927 apparently documented one of the first cases, although others disputed the diagnosis.3*8 If one is to adopt a purist’s view of such tumors, then only those histologically arising from Stensen’s duct are permitted 1.11.12 The clinical presentation in our case of a .painless nodule located near the orifice of Stensen’s duct is in keeping with the reported cases. Further, in our case, that a zone of transition from the columnar epithelium of Stensen’s duct to the carcinomatous region was identified and that the gland proper was not involved are consistent with these types of tumors. These findings fulfill the criteria previously set forth that PCSD should be distinct from both the gland parenchyma and the buccal mucosa and, histologically, the lesion should be an epidermoid, mucoepidermoid, or undifferentiated cancer.‘.‘2 This differential diagnosis of squamous cell carcinoma,
82
FIGURE 1. Left Stensen’s duct with high-grade intraluminal MEC (hematoxylin-eosin, original magnification ~6.6).
CARCINOMA OF STENSEN’S
DUCT
FIGURE 3. Interlobular duct of right parotid gland with zone of transition to an area of moderate epithelial dysplasia (hematoxylin-eosin, original magnification X33).
MEC, and undifferentiated carcinoma is in keeping with the theories of both Eversole and Batsakis regarding salivary gland histogenesis.25,26 By virtue of their rarity, the treatment of PCSD must be established on an individual basis with general principles set forth by Owens et al as well as Clairmont.9,10 These authors advocate surgical excision with an extraoral approach, as done in our case, to obtain adequate exposure, adequate margins, and identification of the facial nerve. Frechette et al set forth criteria of treatment based on clinical staging and advocate radiation therapy as an adjunctive procedure.‘* In our case, consideration was given to postoperative radiotherapy to the left cheek. However, due to the fact that the carcinoma was localized to the duct with no involvement of the parotid gland, we elected not to deliver postoperative radiation. The second striking aspect in our case was the development of in situ neoplasia in the right parotid gland (Fig 3) followed by MEC of the residual right parotid gland (Fig 4). Trumbull and Frazell reported
that 1% of patients with salivary gland tumors have involvement of more than one gland or multiple foci in the same gland.21 A more recent study by Katz and Preston-Martin reported that 13% of their patients had multiple salivary gland tumors; these patients had a history of previous irradiation to the head and neck, as did our case.17 The concept of head and neck neoplasms arising in areas previously irradiated is not novel. Duffy and Fitzgerald first described the relationship between thymic irradiation and development of thyroid carcinoma.24 Rice et al in 1976 cited 23 cases of malignant salivary gland tumors associated with postirradiation and also reported one additional case following radiation treatment for acne.*’ These authors state that MEC is the most frequent lesion to develop and also that a latent period of 20 years or more is required for neoplastic development. Schneider et al in 1986 reviewed 2,561 patients given external radiation before 15 years of age and found that 65 of these patients had developed sali-
FIGURE 2. Higher-power view of Figure 1 showing transition from ductal epithelium to a high-grade MEC (hematoxylin-eosin, original magnification X33).
FIGURE 4. Right parotid gland with high-grade MEC similar to the tumor that arose in the left Stensen’s duct (hematoxylineosin, original magnification x 132).
83
HAAR ET AL
Table 1.
Primary Carcinoma of Stensen’s Duct Location
Author(s)
Age
Sex
Goforth (1927)
60
F
Left distal duct
Lyall & Golcomb (1954) Peracchio (1958)
40
M
51
Maisel et al (1959)
Diagnosis
Treatment
Outcome
Local excision and radium
Died 1% yr later with clinical metastases
Left posterior to orifice
Anaplastic squamous cell carcinoma Squamous cell carcinoma
7,900 rad after biopsy
Free of disease 18 mo follow-up
F
Left, 1.5 cm from orifice
Squamous cell carcinoma
Free of disease 4 mo after therapy
53
M
Right, 1 cm posterior to orifice
Squamous cell carcinoma
Gaisforth et al (1965) Gaisforth et al (1965) Gaisforth et al (1965) Vigorita et al (1980) Owens et al (1982)
55
F
Left duct
Mucoepidermoid carcinoma
2,828 rad plus 12 (2 mg) radon needles Radical excision followed by dissection and radical excision of maxillary bone and soft palate Parotidectomy and neck dissection
25
F
Right buccal
Mucoepidermoid carcinoma
No recurrence follow-up
?
M
Right duct
Mucoepidermoid carcinoma
Superficial parotidectomy and radiation Parotidectomy
82
M
Left, 1 cm from orifice
Squamous cell carcinoma
Free of disease 12 mo later
62
M
Right duct
Squamous cell carcinoma
Clairmont et al ( 1979) Frechette et al (1984) Present case
53
F
Right duct
Mucoepidermoid carcinoma
Left parotidectomy, radical neck dissection Parotidectomy and 4,540 rad to neck and 5,000 rad to primary site Parotidectomy
24
M
Left duct
Undifferentiated carcinoma
Not stated
57
M
Left near orifice
Mucoepidermoid carcinoma
Superficial parotidectomy and 6,240 rad Superficial parotidectomy
vary tumors and had an increased frequency of thyroid neoplasms. I9 Palmer et al, in their study of 662 patients with salivary gland tumors, found that 12 of the patients received radiation for acne.23 Swelstad et al documented 13 salivary gland neoplasms in 18 patients with a history of prior radiation to the head and neck; 7 of these patients received radiation for facial acne.** Previous head and neck radiation in childhood and infancy is a factor in the pathogenesis of neoplasms involving the thyroid and also the salivary glands, with a latent period ranging from 10 to 25 years.‘6-23 However, neither the duration of the risk nor the role of chronic sialadenitis in the development of salivary gland carcinoma following irradiation have been defined. References 1. Batsakis JC (ed): Tumors of the head and neck. Baltimore, MD, Williams & Wilkins, 1979, p 45
Free of disease 20 mo later
Died of recurrence in postoperative period 18 mo
Follow-up not stated
Free of disease
Not stated
No recurrence
2. Goforth JL: Carcinoma developing in the parotid (Stensen’s) duct. Am J Med Sci 173:624, 1927 3. Figi FA, Rowland WD: Primary tumors of Stensen’s and Wharton’s ducts. Arch Otolaryngol40:175, 1944 4. Lyall D, Golcomb FM: Carcinoma of Stensen’s duct. Ann Surg 139:364, 1954 5. Beyer T, Blair J, Hamilton P: Adenocarcinoma of the parotid duct. Arch Otolaryngol 63: 196, 1956 6. Peracchio RL: Primary squamous cell carcinoma of Stensen’s duct. Oral Surg 11:123, 1958 7. Maisel B, Pearce C, Connolly J, et al: Carbon-black carcinoma of Stensen’s duct. Arch Surg 78:331, 195!) 8. Gaisforth JC, Hanna D, Sotereanos G: Primary (cancer of Stensen’s duct. Arch Otolaryngol 82:45, 1965 9. Clairmont AA, Hanna D, Anderson V: Carcinoma of Stensen’s duct. Ann Plast Surg 2:158, 1979 10. Owens 0, Fligiel A, Ward P: Primary carcinoma. of Stensen’s duct. Otolaryngol 90:671, 1982 11. Vigorita V, Huvos A, Gerold F: Squamous cell carcinoma of Stensen’s duct. Head Neck Surg 2:513, 1980 12. Frechette C, Demetris A, Barnes E, et al: Primary carcinoma of Stensen’s duct. J Surg Oncol 27: 1, 1984 13. Carpenter R, Watkins R, Thomas J: Primary carcinoma of Stensen’s duct. Br J Surg 73:926, 1986 14. Wolfe S: Salivary duct carcinoma. Arch Otolarygol 101:398, 1975
84
HYPOPROTHROMBINEMIA:
15. Fayemi A, Toker C: Salivary duct carcinoma. Arch Otolaryngol 99:366, 1974 16. Schneider A, Shore-Freedman E, Weinstein RA: Radiation induced thyroid and other head and neck tumors. J Clin Endocrinol Metah 63: 107, 1986 17. Katz A, Preston-Martin S: Salivary gland tumors and previous radiotherapy to the head or neck. Am J Surg 147:345, 1984
21. 22.
23.
18. Maxon H, Saengev E, Buncher C, et al: Radiation associ-
ated carcinoma of the salivary glands. Ann Otol Rhino1 Laryngol90: 107, 1981 19. Schneider A, Favus M, Stachura M: Salivary gland neoplasms as a late consequence of head and neck irradiation. Ann Intern Med 87:160, 1977 20. Rice D, Batsakis J, McClatchey K: Post irradiation malig-
J Oral Maxillofac 49:84-87.
24. 25. 26.
REPORT OF A CASE
nant salivary gland tumor. Arch Otolaryngol 102:699, 1976 Tumbull A, Frazell E: Multiple tumors of major salivary glands. Am J Surg 118:434,-1969 Swelstad J. Scanlon E. Oviedo M. et al: Irradiation induced polyglandular neoplasia of the head and neck. Am J Surg 135:820, 1978 Palmer J, Mustard R, Simpson W: Irradiation as an etiologic fact in tumors of the thyroid, parathyroid, and salivary glands. Can J Surg 23:39, 1980 Duffy B, Fitzgerald P: Thyroid cancer in childhood and adolescence. J Clin Endocrinol 10:1296, 1950 Eversole L: Histogenic classification of salivary tumors. Arch Path01 92:433, 1971 Regezi J, Batsakis J: Histogenesis of salivary gland neoplasms. Otol Clin 10:297, 1977
Surg
1991
Hypoprothrombinemia (Factor II Deficiency): Report of a Case and Review of Literature ABDOLLAH RAHIMI, DDS,* RAWLE PHILBERT, DDS,t J. HAMIL WILLOUGHBY, DDS,$ AND DUDLEY S. JACKMAN, DMD§ Hypoprothrombinemia is a rare deficiency in the coagulation protein cascade. Only 10 cases of this inherited coagulation disorder have been reported in the literature. This report documents the management of a 45-year-old Hispanic man with known hypoprothrombinemia who presented to our service for multiple surgical odontectomies. Review of the Literature Quick, Piscotta, and Hussey reported the first case of factor II deficiency in 1955. ’ This disorder of prothrombin synthesis is categorized as hypopro-
Received from the Department of Oral and Maxillofacial Surgery, Harlem Hospital Center, New York. * Chief Resident. t Third-Year Resident. $ Chief. 5 Associate Chief. Address correspondence and reprint requests to Dr Willoughby: Department of Oral and Maxillofacial Surgery, Harlem Hospital Center, Martin Luther King Pavilion, Room 6215, 506 Lenox Ave, New York, NY 10037. 0 1991 American
Association
of Oral and Maxillofacial Sur-
geons 0278-2391/91/4901-0015$3.00/O
thrombinemia or dysprothrombinemia.’ Hypoprothrombinemia denotes a decrease in the synthesis or the absence of synthesis of a normal prothrombin polypeptide chain and a decreased level of factor II is described as the antigen. 3-5Dysprothrombinemia production of an abnormal prothrombin polypeptide chain with decreased or absent biological activity and near-normal levels of factor II antigen.3T6 Since 1970, 10 cases of hereditary hypoprothrombinemia documented by chemical or immunochemical assays have been reported.3Y4 The majority of the cases of hereditary hypoprothrombinemia have been among individuals of Mediterranean descent.3T5 The frequency of the hypoprothrombinemia gene has been estimated as 2110,000 in mainland Italian and Hispanic populations. Dysprothrombinemia is an autosomal recessive disorder. Patients with the heterozygous form have approximately 50% of normal levels of prothrombin and do not have bleeding manifestations. Bleeding manifestations have been reported with prothrombin levels ranging from 25% to less than 2%, with most reported cases in the range of 5% to 16%.2V3 In hypoprothrombinemia, the prothrombin time (PT) may or may not be prolonged and the test is not a very sensitive indicator of the amount of prothrombin available for coagulation. Levels of 1% to