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Gigantic ameloblastoma of the mandible complicating hypoproteinemia: case report
Gigantic ameloblastoma of the mandible complicating hypoproteinemia: case report Yoshiya Ueyama, Gohichi Tsukamoto, Tomohiro Matsumura
Department of Oral & Maxillofacial Surgery H (Head." Prof. T. Matsumura DDS., PhD.), Okayama University Dental School 2-5-1 Shikata-Cho Okayama 700 Japan
SUMMARY. A case of gigantic ameloblastoma of the mandible complicating hypoproteinemia is reported. The patient, a 73-year-old male, had refused a surgical procedure on an ameloblastoma for 13 years. By the time the tumor had increased in size and fistulas from it had formed, hypoproteinemia and generalized edema had occurred. The tumor was removed when the serum total protein level had recovered to about 5 g[dl following the administration of a plasma protein preparation. After the operation, hypoproteinemia and edema clearly improved. Hypoproteinemia is thought to be caused by leakage of plasma or occasional bleeding through the oral fistulas of ameloblastoma, and in this patient's case, poor nutrition because of his masticatory and swallowing difficulties.
of whole blood, plasma and albumin was carried out, but the patient rejected the idea of a surgical procedure twice whenever his general condition had somewhat improved. However, he was finally admitted to our hospital on September 29, 1987 because his condition had become worse. Physical examination showed a poorly nourished, and severely anemic-looking man, but fully conscious He had severe generalized edema, especially on the
INTRODUCTION Ameloblastoma is the most common benign neoplasm originating from odontogenic epithelia. It mainly occurs in the mandibular molar-ramus region in the 20- to 30-year-old age group (Suzuki, 1985). In recent times, ameloblastoma is usually found and treated at an early stage because pantomographic examinations are often needed for dental diagnostic purposes. Consequently, it is quite rare to find such a gigantic ameloblastoma as in our reported case, which grew enormously, developing hypoproteinemia as a secondary complication. The development and treatment of a gigantic ameloblastoma with hypoproteinemia is reported. CASE REPORT A 73-year-old man had first noticed a swelling on the left side of the mandible in 1975, but did not seek medical attention because the lesion was painless and grew only slowly. In 1981, a biopsy was performed at another hospital, and the tumor was diagnosed histologically as an ameloblastoma. He rejected any surgical procedure because of a fear of surgery. Since then, the tumor grew slowly and generalized edema occurred. In September 1985 he was referred to our clinic for treatment of the ameloblastoma, by our internal medicine department. A medical examination revealed severe anemia and hypoproteinemia. Though hypoproteinemia is considered to be most often caused by protein-losing gastroenteropathy, the nephrotic syndrome and diabetes, clinical examination and laboratory data did not suggest these illnesses. Finally, we concluded that his hypoproteinemia was mainly caused by the leakage of plasma protein from the fistulas. In addition to exudate, bleeding occasionally occurred from the fistulas. Transfusion
Fig. 1 -Preoperative facialappearance. 47
48
Journal of Cranio Maxillo-Facia1 Surgery
Continuous leakage of exudate via the fistulas was observed• Because the tongue was displaced upwards and backwards the patient could hardly breathe and swallow• The pantomograph revealed that the mandibular bone was defective from the left coronoid process to the right angle of mandible• Some nodular formations in the mass were seen on the computed tomograph (Fig. 2). Because his hypoproteinemia was severe, packed red blood cells and plasma protein were administered before the operation (Fig. 3). Following the increase of TP, the edema disappeared to some degree and the circumference of his abdomen decreased from 96 cm to 76 cm. On November 13 1987, under general anesthesia after tracheostomy, the tumor was extirpated by mandibular dissection followed by immediate reconstruction with a single A-O plate (Mathis Corporation, Switzerland)• Blood loss was 1350 ml, so packed red blood cells and single donor fresh-frozen plasma (FFP) were transfused (Fig. 3). On the next day the patient went into heart failure and he was sent to the ICU at Okayama University Medical Hospital, for four days. The tumor removed was 90 × 100 x 75 mm in size and its total weight was 435 g. Macroscopically, the tumor was well circumscribed, and necrotic tissue in the centre of the tumor extended to the oral fistulas• Histologically, the mandible was replaced with tumor cells. The greater part of tumor showed a plexiform ameloblastoma (Fig. 4). In some parts of the central area of the tumor nests of granular cells were seen. In spite of hypoproteinemia before the operation,
Fig. 2 - Preoperative CT showing some nodular formations in the tumor mass (arrows).
abdomen and legs. He was hardly able to walk by himself• The chest X-ray revealed severe lung edema• Extraorally, the gigantic swelling, which measured 11 x 11 x 14cm, extended from the left mandibular angle to the right mental foramen region (Fig. 1). Intraorally, the mouth floor was raised from the left retromolar to the right molar region and the mucous membrane was distended by the tumor expansion• One fistula, which was approx 40 mm at the greatest diameter, opened bilaterally in the lower molar area.
TP AIb (g/dl)
Ope.
8"
........................ .. ...... ......•..... ..........•.~,,~ • [ Albu.min-.al.p!a7.5OralP!asm.an.a!.e. !1.37.0.m.I..'~.::iii::i::iii::i:ii:i::i::i::i::i:::::i ::::::::::::::::::::::::::::::::: i iii!iiii::i::!i ::::::::ii!i:i: :i!~:: ~::i::i::i::::!i::l
765 4 3-
2'
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:::::::::::::::::::::::::::::::::::::: : iii iiiiiii{iiiiiiiii i ii i ii;ii! ii ii ::i iiii ii::i!i!i i;iii . : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : ::::::::::::::::::::::::::::::::::::::::::::::::::::::::::::::::::::::::::: ..............."
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' ............" " " : ~ -
,,..,,,....J
. . . . . . . . . " " ................ - - ~ 2 ; ; " " " "
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~.
.~,---~,,
I
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-"
° •
•
"\.,,.,,,.. . . . /
"10
1
"~'~ ./f/" ",~ Traisfusion* 18oomlL_/ 10
*PackedRed BloodCells
5
0
48oml 1'1
11 13
Fig. 3 - Kinetics of the preoperative and postoperative values of TP, Alb, Hgb.
12
Month
Gigantic ameloblastoma of the mandible complicating hypoproteinemia: case report
Fig. 4 - Photomicrograph of the extirpated tumor shows the typical pattern of plexiform ameloblastoma.
the healing of the operation wound was good. Jaw and tongue movements were obtained to some degree. Though the plasma protein preparation was not administered after the operation, hypoproteinemia and generalized edema have improved remarkably. The patient was discharged from our hospital on December 30, 1988. There has been no evidence of recurrence up to now. DISCUSSION A few cases of ameloblastoma with hypoproteinemia have been reported and the cause of hypoproteinemia has been discussed. It has been reported that the cystic wall has the qualities of semipermeable membrane and the exudate in the cyst leaks through the fistulas (Kubo et al., 1977; Osakiet al., 1985; Yoshikawa et al., 1990). The serum protein fraction was determined twice before the operation indicating an extreme reduction of TP and Alb. In the globulin fraction, alpha-globulin and beta-globulin values were almost the same before and after the operation when these data were compared with the absolute quantity. Alb. values became normal after the operation. These findings certainly made us believe that the Alb. that leaked through the fistulas came from the penetration of low weight proteins through the cyst wall which had behaved as a selective permeable membrane for proteins depending on their molecular weight (Kaneko, 1972). But when the ameloblastoma exudate was compared with blood serum, the protein fraction of the exudate was relatively similar to that of the serum. The exudate was mixed with saliva judging from the high amylase values, but there was a small amount of globulin. These results then, showed that proteins might probably pass nonselectively through the cyst wall. It is still unclear if the cyst wall is permeable to proteins depending on their molecular weight, acting as a selective or nonselective membrane. However, it is obvious that continuous leakage of exudate might possibly cause hypoproteinemia; moreover intermittent bleeding through the fistulas and low nutrition were also considered as a cause. Kubo et al. (1977) reported that among 41 cases of amelo-
49
blastoma, 16 had a fistula and of these only 2 cases were accompanied by hypoproteinemia. These two cases were gigantic ameloblastomas. In spite of there being no administration of plasma protein preparation after the removal of the tumor, the data increased significantly and returned to the normal range. This data strongly suggested that plasma protein leaked through the oral fistulas of the tumor. From other reports (Kubo et al., 1977; Osaki et al., 1985; Yoshikawa et al., 1990) and our case, we can conclude that an ameloblastoma which grows excessively and has fistulas might complicate hypoproteinemia. A surgical procedure is generally indicated on condition that TP is over 6 g/dl, but we performed it when TP was 5.09 g/dl. In the past the results of operations at 4.5 to 5.0 g/dl of TP have been reported and they have been generally favorable (Kubo et al., 1977; Petriella, 1974). As long as a fistula exists, it is very difficult to improve TP to the normal range and even if a lot of plasma protein preparation is administered, there is a danger that sudden massive bleeding will occur (Yoshikawa et al., 1990). Thus, it is suggested that surgical removal of a tumor should be performed as soon as the TP level is elevated to about 5 g/dl. The patient received internal medicine care before the operation since an ECG showed myocardial injury. We made an effort not to administer infusions excessively during and after the operation, but it was necessary to transfuse blood and albumin due to excessive bleeding. Heart failure occurred on the day after the operation. Consequently the major reason was considered to be hypervolemia caused by transfusion, another reason was the influence of cardiac stress caused by the whole body and lung edema before the operation. References Kaneko, L : Biochemical studies on cystic fluid of the jaw. Jap. J. Oral Surg. 18 (1972) ~ 1 4 . Kubo, K., H. Tashiro, M. Oka, M. Shikata, Y. Otsuki: Ameloblastoma of the mandible with hypoproteinemia. Jap. J. Oral Surg. 23 (1977) 99-104 1977. Osaki, T., K. Ryoke, T. Nagami, T. Ogawa, T. Hamada." Ameloblastoma with hypoproteinemia due to protein leakage. Int. J. Oral Surg. 14 (1985) 302-306. Petriella, V. M., P. N. Rogow, E. Baden, A. C. Williams: Gigantic ameloblastoma of the mandible: report of case. J. Oral Surg. 32 (1974) 44-49. Suzuki, A. : Odontogenic tumors-pathological aspects. Jpn. Pathol. Clin. Med 3 (1985) 869-877. Yoshikawa, F., K. Higuehi, M. Tsujino, T. Moriyama, T. Kawamoto, T. Sugiyama, M. Sakuta: Mandibular ameloblastoma complicating severe hypoproteinemia: report of a case. Jpn. J. Oral Maxillofac. Surg. 36 (1990) 146-147. Dr Y. Ueyama, DDS, PhD Dept of Oral and Maxillofacial Surgery II Okayama University Dental School 2-5-1 Shikata-Cho, Okayama 700 Japan Paper received: 13 December, 1993 Accepted: 4 July 1994
Department of Oral & Maxillofacial Surgery H (Head." Prof. T. Matsumura DDS., PhD.), Okayama University Dental School 2-5-1 Shikata-Cho Okayama 700 Japan
SUMMARY. A case of gigantic ameloblastoma of the mandible complicating hypoproteinemia is reported. The patient, a 73-year-old male, had refused a surgical procedure on an ameloblastoma for 13 years. By the time the tumor had increased in size and fistulas from it had formed, hypoproteinemia and generalized edema had occurred. The tumor was removed when the serum total protein level had recovered to about 5 g[dl following the administration of a plasma protein preparation. After the operation, hypoproteinemia and edema clearly improved. Hypoproteinemia is thought to be caused by leakage of plasma or occasional bleeding through the oral fistulas of ameloblastoma, and in this patient's case, poor nutrition because of his masticatory and swallowing difficulties.
of whole blood, plasma and albumin was carried out, but the patient rejected the idea of a surgical procedure twice whenever his general condition had somewhat improved. However, he was finally admitted to our hospital on September 29, 1987 because his condition had become worse. Physical examination showed a poorly nourished, and severely anemic-looking man, but fully conscious He had severe generalized edema, especially on the
INTRODUCTION Ameloblastoma is the most common benign neoplasm originating from odontogenic epithelia. It mainly occurs in the mandibular molar-ramus region in the 20- to 30-year-old age group (Suzuki, 1985). In recent times, ameloblastoma is usually found and treated at an early stage because pantomographic examinations are often needed for dental diagnostic purposes. Consequently, it is quite rare to find such a gigantic ameloblastoma as in our reported case, which grew enormously, developing hypoproteinemia as a secondary complication. The development and treatment of a gigantic ameloblastoma with hypoproteinemia is reported. CASE REPORT A 73-year-old man had first noticed a swelling on the left side of the mandible in 1975, but did not seek medical attention because the lesion was painless and grew only slowly. In 1981, a biopsy was performed at another hospital, and the tumor was diagnosed histologically as an ameloblastoma. He rejected any surgical procedure because of a fear of surgery. Since then, the tumor grew slowly and generalized edema occurred. In September 1985 he was referred to our clinic for treatment of the ameloblastoma, by our internal medicine department. A medical examination revealed severe anemia and hypoproteinemia. Though hypoproteinemia is considered to be most often caused by protein-losing gastroenteropathy, the nephrotic syndrome and diabetes, clinical examination and laboratory data did not suggest these illnesses. Finally, we concluded that his hypoproteinemia was mainly caused by the leakage of plasma protein from the fistulas. In addition to exudate, bleeding occasionally occurred from the fistulas. Transfusion
Fig. 1 -Preoperative facialappearance. 47
48
Journal of Cranio Maxillo-Facia1 Surgery
Continuous leakage of exudate via the fistulas was observed• Because the tongue was displaced upwards and backwards the patient could hardly breathe and swallow• The pantomograph revealed that the mandibular bone was defective from the left coronoid process to the right angle of mandible• Some nodular formations in the mass were seen on the computed tomograph (Fig. 2). Because his hypoproteinemia was severe, packed red blood cells and plasma protein were administered before the operation (Fig. 3). Following the increase of TP, the edema disappeared to some degree and the circumference of his abdomen decreased from 96 cm to 76 cm. On November 13 1987, under general anesthesia after tracheostomy, the tumor was extirpated by mandibular dissection followed by immediate reconstruction with a single A-O plate (Mathis Corporation, Switzerland)• Blood loss was 1350 ml, so packed red blood cells and single donor fresh-frozen plasma (FFP) were transfused (Fig. 3). On the next day the patient went into heart failure and he was sent to the ICU at Okayama University Medical Hospital, for four days. The tumor removed was 90 × 100 x 75 mm in size and its total weight was 435 g. Macroscopically, the tumor was well circumscribed, and necrotic tissue in the centre of the tumor extended to the oral fistulas• Histologically, the mandible was replaced with tumor cells. The greater part of tumor showed a plexiform ameloblastoma (Fig. 4). In some parts of the central area of the tumor nests of granular cells were seen. In spite of hypoproteinemia before the operation,
Fig. 2 - Preoperative CT showing some nodular formations in the tumor mass (arrows).
abdomen and legs. He was hardly able to walk by himself• The chest X-ray revealed severe lung edema• Extraorally, the gigantic swelling, which measured 11 x 11 x 14cm, extended from the left mandibular angle to the right mental foramen region (Fig. 1). Intraorally, the mouth floor was raised from the left retromolar to the right molar region and the mucous membrane was distended by the tumor expansion• One fistula, which was approx 40 mm at the greatest diameter, opened bilaterally in the lower molar area.
TP AIb (g/dl)
Ope.
8"
........................ .. ...... ......•..... ..........•.~,,~ • [ Albu.min-.al.p!a7.5OralP!asm.an.a!.e. !1.37.0.m.I..'~.::iii::i::iii::i:ii:i::i::i::i::i:::::i ::::::::::::::::::::::::::::::::: i iii!iiii::i::!i ::::::::ii!i:i: :i!~:: ~::i::i::i::::!i::l
765 4 3-
2'
:: :!i!i!i!i?iiiiiiiiiiiiiii ii?i!iiii?i!iiiiii i !iiiiiii!i!i!iiiii!iiiiiiiiiiiii!ii?iii i: .
:::::::::::::::::::::::::::::::::::::: : iii iiiiiii{iiiiiiiii i ii i ii;ii! ii ii ::i iiii ii::i!i!i i;iii . : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : : ::::::::::::::::::::::::::::::::::::::::::::::::::::::::::::::::::::::::::: ..............."
"
~
..............
' ............" " " : ~ -
,,..,,,....J
. . . . . . . . . " " ................ - - ~ 2 ; ; " " " "
.....--" ,..,
Hgb (g/dO
.-...........:.....-.-.-.:.:.;.~,,~...............,...............•...........•............ /
~.
.~,---~,,
I
~"
-"
° •
•
"\.,,.,,,.. . . . /
"10
1
"~'~ ./f/" ",~ Traisfusion* 18oomlL_/ 10
*PackedRed BloodCells
5
0
48oml 1'1
11 13
Fig. 3 - Kinetics of the preoperative and postoperative values of TP, Alb, Hgb.
12
Month
Gigantic ameloblastoma of the mandible complicating hypoproteinemia: case report
Fig. 4 - Photomicrograph of the extirpated tumor shows the typical pattern of plexiform ameloblastoma.
the healing of the operation wound was good. Jaw and tongue movements were obtained to some degree. Though the plasma protein preparation was not administered after the operation, hypoproteinemia and generalized edema have improved remarkably. The patient was discharged from our hospital on December 30, 1988. There has been no evidence of recurrence up to now. DISCUSSION A few cases of ameloblastoma with hypoproteinemia have been reported and the cause of hypoproteinemia has been discussed. It has been reported that the cystic wall has the qualities of semipermeable membrane and the exudate in the cyst leaks through the fistulas (Kubo et al., 1977; Osakiet al., 1985; Yoshikawa et al., 1990). The serum protein fraction was determined twice before the operation indicating an extreme reduction of TP and Alb. In the globulin fraction, alpha-globulin and beta-globulin values were almost the same before and after the operation when these data were compared with the absolute quantity. Alb. values became normal after the operation. These findings certainly made us believe that the Alb. that leaked through the fistulas came from the penetration of low weight proteins through the cyst wall which had behaved as a selective permeable membrane for proteins depending on their molecular weight (Kaneko, 1972). But when the ameloblastoma exudate was compared with blood serum, the protein fraction of the exudate was relatively similar to that of the serum. The exudate was mixed with saliva judging from the high amylase values, but there was a small amount of globulin. These results then, showed that proteins might probably pass nonselectively through the cyst wall. It is still unclear if the cyst wall is permeable to proteins depending on their molecular weight, acting as a selective or nonselective membrane. However, it is obvious that continuous leakage of exudate might possibly cause hypoproteinemia; moreover intermittent bleeding through the fistulas and low nutrition were also considered as a cause. Kubo et al. (1977) reported that among 41 cases of amelo-
49
blastoma, 16 had a fistula and of these only 2 cases were accompanied by hypoproteinemia. These two cases were gigantic ameloblastomas. In spite of there being no administration of plasma protein preparation after the removal of the tumor, the data increased significantly and returned to the normal range. This data strongly suggested that plasma protein leaked through the oral fistulas of the tumor. From other reports (Kubo et al., 1977; Osaki et al., 1985; Yoshikawa et al., 1990) and our case, we can conclude that an ameloblastoma which grows excessively and has fistulas might complicate hypoproteinemia. A surgical procedure is generally indicated on condition that TP is over 6 g/dl, but we performed it when TP was 5.09 g/dl. In the past the results of operations at 4.5 to 5.0 g/dl of TP have been reported and they have been generally favorable (Kubo et al., 1977; Petriella, 1974). As long as a fistula exists, it is very difficult to improve TP to the normal range and even if a lot of plasma protein preparation is administered, there is a danger that sudden massive bleeding will occur (Yoshikawa et al., 1990). Thus, it is suggested that surgical removal of a tumor should be performed as soon as the TP level is elevated to about 5 g/dl. The patient received internal medicine care before the operation since an ECG showed myocardial injury. We made an effort not to administer infusions excessively during and after the operation, but it was necessary to transfuse blood and albumin due to excessive bleeding. Heart failure occurred on the day after the operation. Consequently the major reason was considered to be hypervolemia caused by transfusion, another reason was the influence of cardiac stress caused by the whole body and lung edema before the operation. References Kaneko, L : Biochemical studies on cystic fluid of the jaw. Jap. J. Oral Surg. 18 (1972) ~ 1 4 . Kubo, K., H. Tashiro, M. Oka, M. Shikata, Y. Otsuki: Ameloblastoma of the mandible with hypoproteinemia. Jap. J. Oral Surg. 23 (1977) 99-104 1977. Osaki, T., K. Ryoke, T. Nagami, T. Ogawa, T. Hamada." Ameloblastoma with hypoproteinemia due to protein leakage. Int. J. Oral Surg. 14 (1985) 302-306. Petriella, V. M., P. N. Rogow, E. Baden, A. C. Williams: Gigantic ameloblastoma of the mandible: report of case. J. Oral Surg. 32 (1974) 44-49. Suzuki, A. : Odontogenic tumors-pathological aspects. Jpn. Pathol. Clin. Med 3 (1985) 869-877. Yoshikawa, F., K. Higuehi, M. Tsujino, T. Moriyama, T. Kawamoto, T. Sugiyama, M. Sakuta: Mandibular ameloblastoma complicating severe hypoproteinemia: report of a case. Jpn. J. Oral Maxillofac. Surg. 36 (1990) 146-147. Dr Y. Ueyama, DDS, PhD Dept of Oral and Maxillofacial Surgery II Okayama University Dental School 2-5-1 Shikata-Cho, Okayama 700 Japan Paper received: 13 December, 1993 Accepted: 4 July 1994