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Gigantic ameloblastoma associated with secondary hypoproteinemia
764
GIGANTIC AMELOBLASTOMA WITH HYPOPROTEINEMIA
J Oral Maxillolac Surg 49:764-767,1991
Gigantic Ameloblastoma Associated With Secondary Hypoproteinemia SHIGEKI NAKASATO, DDS, DMSc,* SATORU OKAMURA, DDS,t KEIGO KUDO, DDS, DMSc,+ AND YASUNORI TAKEDA, DDS, PHD§ Because of its slow and asymptomatic growth, many patients with ameloblastoma seek medical treatment only after the lesion has grown remarkably large.1.2 We recently encountered a case of a gigantic ameloblastoma causing severe hypoproteinemia 3-s and anemia/ in a patient who had been aware of the tumor growth for more than 20 years. This article describes preoperative and postoperative findings and the clinical course of this patient. Report of Case The patient was a 39-year-old woman who came to the Department of Dental and Oral Surgery, Iwate Prefectural Central Hospital on November 28, 1988, with a swelling in the left cheek. The left lower molars had been extracted by a general dentist 23 years previously (1965). Because of the poor healing of the extraction wound, she had consulted an oral surgeon in 1968. Although surgical treatment had been recommended at the time, she left the lesion unattended because of her fear of surgery. The left cheek began to swell about 6 years ago. Recently, the swelling became conspicuously large. The patient was obese, with a body weight of 82.8 kg. She complained of mild fatigue. Her extremities were edematous and her face and palpebral conjunctivae were pale. A hard swelling measuring approximately II x 10 x 6 ern was found in the left cheek and mandible (Fig I). The skin over the tumor had a normal texture and was red, but the lesion was nontender. The swelling caused difficulty in closure of the lips on the left side of the mouth and the angle of the mouth sagged.
FIGURE I. View of patient at the first examination showing the asymmetrical swelling of the left cheek.
* Oral Surgeon and Chief, Department of Dental and Oral Surgery, Iwate Prefectural Central Hospital, Morioka. Iwate, Japan. t Resident, Department of Dental and Oral Surgery, Iwate Prefectural Central Hospital, Morioka, Iwate, Japan. :j: Associate Professor, First Department of Oral and Maxillofacial Surgery, School of Dentistry, Iwate Medical University, Morioka, Iwate, Japan. § Assistant Professor, Department of Oral Pathology, School of Dentistry, Iwate Medical University, Morioka, Iwate, Japan. Address correspondence and reprint requests to Dr Kudo: First Department of Oral and Maxillofacial Surgery, School of Dentistry, Iwate Medical University, 1-3-27 Chuo-Dori, Morioka, Iwate 020, Japan. © 1991 American Association of Oral and Maxillofacial Surgeons
0278-2391/91/4907-002153.00/0
FIGURE 2.
Intraoral view of tumor and ulcer (arrow).
765
NAKASATO ET AL
Table 1. Preoperative and Postoperative Results of the Laboratory Examinations
White blood cells Red blood cells Hemoglobin Hematocrit TP
NG FIGURE 3. Panoramic radiograph showing a multilocular radiolucent lesion of the left mandible.
The mouth could be opened only about 20 rnm. The left posterior mandibular alveolar region was displaced lin.gually. A rough-surfaced, elastic swelling was present in the buccal mucosa. The mucosal surface was reddened and bled easily. A 2 x 2.5-cm ulcer was present in the left lower first and second molar region (Fig 2). There was a massive outflow of exudate from this area. Radiographic examination showed almost complete loss of bone from the apex of the left lower canine to the condyle. The lateral wall of the maxillary sinus was compressed. The radiographic findings suggested the expansive growth of an advanced tumor (Fig 3). Magnetic resonance imaging (MRI) disclosed proliferation of the upper portion of the tumor into the temporal fossa. The masseter lateral to the fossa was atrophied. Medial to the fossa there was a cystic component. The MRI also showed streaking as an intermediate signal within the tumor (Fig 4). Marked hypoproteinemia, iron deficiency anemia, and leukocytosis were found by laboratory examination (Table I). These conditions were considered to have resulted from secondary inflammation and the long-term leakage of exudate from the tumor. A high-protein diet, 25% albumin (50 mUd), Protearnin 12X (400 mUd; Tanabe Corp, Japan), and an iron preparation were prescribed, but were almost completely ineffective in decreasing the severing of the symptoms. Early surgical excision was planned after consultation with the anesthesiologists. On January 19, 1989, surgical excision of the tumor was performed under general anesthesia. The tumor was ex-
FIGURE 4. T'l-weighted coronal MRI showing extension of the tumor into the temporal fossa.
Albumin lX.-globulin lX2-globulin l3-globulin 'Y-globulin Na K CI Ca Fe Total iron-binding capacity
Before Operation
After Operation
13.14 x 103 3.87 x IO~ 8.9 g1dL 29.3% 3.1 g1dL 1.03 50.9% 9.5% 17.5% 12.1% 10.0% 143.2 mEq/L 3.73 mEq/L 109.4 mEq/L 7.0 mg/dl, 6 ug/dl,
3.93 X 103 4.35 x IO~ 12.5 g1dL 37.5% 6.4 g1dL 1.51 60.2% 3.2% 7.9% 8.7% 20.0% 142.8 mEq/L 4.1 mEq/L 110.4 mEq/L 8.9 mg/dl,
113 ug/dl,
145 ug/dl,
215 fLgldL
posed through incisions from the left temporal region to the anterior auricular region, at the posterior region of the mandibular angle, and from the submandibular region to the right mentum. The mandible was resected distally from the left lateral incisor and the tumor was excised en bloc. The specimen weighed 386 g. The defect was temporarily reconstructed using a titanium mandibular reconstruction plate with a condyle attached (Wiirzburg System; Leibinger Corporation, Germany) (Fig 5). Because about 4,000 mL of blood was lost during the long operation, blood transfusion was carried out. During the operation, no abnormalities were encountered except slight blood pressure changes. The wound healed uneventfully and no evidence of postoperative infection was detected. The laboratory abnormalities noted earlier rapidly improved postoperatively (Table I). The histologic diagnosis of the lesion was plexiform ameloblastoma. No atypical findings were seen in individual tumor cells. Interstitial cysts were located throughout the large cyst. A large volume of fluid was retained in these cysts (Fig 6).
766
GIGANTIC AMELOBLASTOMA WITH HYPOPROTEINEMIA
FIGURE 5. Panoramic radiograph showing reconstruction plate 3 months after surgery.
In a radiograph made 1.5 years postoperatively, no sign of recurrence was detected in either the articular fossa or mandibular stump. Although the plate was positioned somewhat lateral to the mandibular ramus. bone resorption was not found at the site of fixation . The left cheek and mandibular body were slightly depressed and a sensory deficit existed in the lower lip. However. mouth opening had increased to about 40 mm. The displacement
of the mandible on the side of the lesion was slight. Because teeth still remained on the unaffected side. relatively good occlusal function could be restored by treatment of the _remaining teeth after surgery. Although no denture was fitted to the affected side, oral function was little disturbed. the patient's daily routine could be achieved, and she was quite satisfied with the results (Fig 7).
FIGURE 6. Photomicrographs showing (8) plexiform ameloblastoma (hematoxylin-eosin, original magnification x200) and (A) formation of large stromal cysts (hematoxylin-eosin, original magnification x40).
FIGURE 7. Relatively symmetrical facial appearance at 18 months after surgery
767
NAKASATO ET AL
Discussion The introduction of the panoramic radiograph in routine dental practice has contributed to the earlier detection and treatment of ameloblastoma. However, if a lesion is left unattended for a long time because of a patient's fear of invasive surgery, it can grow remarkably large. This can be followed by the leakage of cystic fluid from the lesion and may result in the development of secondary hypoproteinemia, as found in this case. Four cases of ameloblastoma associated with hypoproteinemia have been reported.l? The histologic diagnosis in these four cases were either follicular 'S or reticular':" type of ameloblastoma. Large ameloblastomas can cause occlusal abnormality and difficulty with food ingestion.P? Patients also may be at risk for hemorrhage due to ulceration and can develop symptoms of anemia. J Furthermore, the general condition of the patient can deteriorate and life may be endangered by pulmonary edema.' The hypoproteinemia can sometimes be considerably improved by administration of protein and albumin preparations, and tissue coagulants':" can be used for hemorrhage, but this does not substitute for excision of the tumor.':" Moreover, the patient described in this article almost totally failed to respond to medication due to the massive leakage of intracystic fluid. The wall of an odontogenic cyst permits proteins
to pcrmcate.P'? Because the molecular weight of albumin is less than that of globulin, it probably passes through the membrane and migrates more easily into the cystic cavity." As a mechanism for the development of hypoproteinemia in gigantic cystic ameloblastoma, two factors are presumed to be involved: the cyst wall acting as a semipermeable membrane and the leakage of intracystic fluid directly through an opening formed in the cyst walI. 3 •5 In this case, the fluid that leaked through the ulcer in the cyst wall flowed so profusely that it was possible to continuously aspirate it using a bougie, thereby causing the hypoproteinemia.
References I. Masuyarna Y, Shiota K: Extensive ameloblastoma of the mandible. Jpn J Oral Surg 2:4, 1956 2. Petriella VI\I: Gigantic ameloblastoma of the mandible. J Oral Surg 32:44, 1974 3. Kubo K, Yasuhiro H, Oka 1\1, et al: Ameloblastoma of the mand ible with hypoproteinemia . Jpn J Oral Surg 23:291, 1977 4. Osaki T, Ryoke T: Amelobl astoma with hypoproteinemia due to protein leakage. Int J Oral Surg 14:302, 1985 5. Yoshikawa F, Higuchi K, Tsujino M, et al: Mandibular ameloblastoma urnplicating severe hypoproteinemia: Report of a case. Jpn J Oral Maxillofac Surg 36:589, 1990 6. Toller PA: Protein sub stances in odontogenic cyst fluid. Br Dent J 128:317, 1970 7. Kaneko I: Biochemic al studies on cystic fluid of the jaw. Jpn J Oral Surg 18:2, 1972
GIGANTIC AMELOBLASTOMA WITH HYPOPROTEINEMIA
J Oral Maxillolac Surg 49:764-767,1991
Gigantic Ameloblastoma Associated With Secondary Hypoproteinemia SHIGEKI NAKASATO, DDS, DMSc,* SATORU OKAMURA, DDS,t KEIGO KUDO, DDS, DMSc,+ AND YASUNORI TAKEDA, DDS, PHD§ Because of its slow and asymptomatic growth, many patients with ameloblastoma seek medical treatment only after the lesion has grown remarkably large.1.2 We recently encountered a case of a gigantic ameloblastoma causing severe hypoproteinemia 3-s and anemia/ in a patient who had been aware of the tumor growth for more than 20 years. This article describes preoperative and postoperative findings and the clinical course of this patient. Report of Case The patient was a 39-year-old woman who came to the Department of Dental and Oral Surgery, Iwate Prefectural Central Hospital on November 28, 1988, with a swelling in the left cheek. The left lower molars had been extracted by a general dentist 23 years previously (1965). Because of the poor healing of the extraction wound, she had consulted an oral surgeon in 1968. Although surgical treatment had been recommended at the time, she left the lesion unattended because of her fear of surgery. The left cheek began to swell about 6 years ago. Recently, the swelling became conspicuously large. The patient was obese, with a body weight of 82.8 kg. She complained of mild fatigue. Her extremities were edematous and her face and palpebral conjunctivae were pale. A hard swelling measuring approximately II x 10 x 6 ern was found in the left cheek and mandible (Fig I). The skin over the tumor had a normal texture and was red, but the lesion was nontender. The swelling caused difficulty in closure of the lips on the left side of the mouth and the angle of the mouth sagged.
FIGURE I. View of patient at the first examination showing the asymmetrical swelling of the left cheek.
* Oral Surgeon and Chief, Department of Dental and Oral Surgery, Iwate Prefectural Central Hospital, Morioka. Iwate, Japan. t Resident, Department of Dental and Oral Surgery, Iwate Prefectural Central Hospital, Morioka, Iwate, Japan. :j: Associate Professor, First Department of Oral and Maxillofacial Surgery, School of Dentistry, Iwate Medical University, Morioka, Iwate, Japan. § Assistant Professor, Department of Oral Pathology, School of Dentistry, Iwate Medical University, Morioka, Iwate, Japan. Address correspondence and reprint requests to Dr Kudo: First Department of Oral and Maxillofacial Surgery, School of Dentistry, Iwate Medical University, 1-3-27 Chuo-Dori, Morioka, Iwate 020, Japan. © 1991 American Association of Oral and Maxillofacial Surgeons
0278-2391/91/4907-002153.00/0
FIGURE 2.
Intraoral view of tumor and ulcer (arrow).
765
NAKASATO ET AL
Table 1. Preoperative and Postoperative Results of the Laboratory Examinations
White blood cells Red blood cells Hemoglobin Hematocrit TP
NG FIGURE 3. Panoramic radiograph showing a multilocular radiolucent lesion of the left mandible.
The mouth could be opened only about 20 rnm. The left posterior mandibular alveolar region was displaced lin.gually. A rough-surfaced, elastic swelling was present in the buccal mucosa. The mucosal surface was reddened and bled easily. A 2 x 2.5-cm ulcer was present in the left lower first and second molar region (Fig 2). There was a massive outflow of exudate from this area. Radiographic examination showed almost complete loss of bone from the apex of the left lower canine to the condyle. The lateral wall of the maxillary sinus was compressed. The radiographic findings suggested the expansive growth of an advanced tumor (Fig 3). Magnetic resonance imaging (MRI) disclosed proliferation of the upper portion of the tumor into the temporal fossa. The masseter lateral to the fossa was atrophied. Medial to the fossa there was a cystic component. The MRI also showed streaking as an intermediate signal within the tumor (Fig 4). Marked hypoproteinemia, iron deficiency anemia, and leukocytosis were found by laboratory examination (Table I). These conditions were considered to have resulted from secondary inflammation and the long-term leakage of exudate from the tumor. A high-protein diet, 25% albumin (50 mUd), Protearnin 12X (400 mUd; Tanabe Corp, Japan), and an iron preparation were prescribed, but were almost completely ineffective in decreasing the severing of the symptoms. Early surgical excision was planned after consultation with the anesthesiologists. On January 19, 1989, surgical excision of the tumor was performed under general anesthesia. The tumor was ex-
FIGURE 4. T'l-weighted coronal MRI showing extension of the tumor into the temporal fossa.
Albumin lX.-globulin lX2-globulin l3-globulin 'Y-globulin Na K CI Ca Fe Total iron-binding capacity
Before Operation
After Operation
13.14 x 103 3.87 x IO~ 8.9 g1dL 29.3% 3.1 g1dL 1.03 50.9% 9.5% 17.5% 12.1% 10.0% 143.2 mEq/L 3.73 mEq/L 109.4 mEq/L 7.0 mg/dl, 6 ug/dl,
3.93 X 103 4.35 x IO~ 12.5 g1dL 37.5% 6.4 g1dL 1.51 60.2% 3.2% 7.9% 8.7% 20.0% 142.8 mEq/L 4.1 mEq/L 110.4 mEq/L 8.9 mg/dl,
113 ug/dl,
145 ug/dl,
215 fLgldL
posed through incisions from the left temporal region to the anterior auricular region, at the posterior region of the mandibular angle, and from the submandibular region to the right mentum. The mandible was resected distally from the left lateral incisor and the tumor was excised en bloc. The specimen weighed 386 g. The defect was temporarily reconstructed using a titanium mandibular reconstruction plate with a condyle attached (Wiirzburg System; Leibinger Corporation, Germany) (Fig 5). Because about 4,000 mL of blood was lost during the long operation, blood transfusion was carried out. During the operation, no abnormalities were encountered except slight blood pressure changes. The wound healed uneventfully and no evidence of postoperative infection was detected. The laboratory abnormalities noted earlier rapidly improved postoperatively (Table I). The histologic diagnosis of the lesion was plexiform ameloblastoma. No atypical findings were seen in individual tumor cells. Interstitial cysts were located throughout the large cyst. A large volume of fluid was retained in these cysts (Fig 6).
766
GIGANTIC AMELOBLASTOMA WITH HYPOPROTEINEMIA
FIGURE 5. Panoramic radiograph showing reconstruction plate 3 months after surgery.
In a radiograph made 1.5 years postoperatively, no sign of recurrence was detected in either the articular fossa or mandibular stump. Although the plate was positioned somewhat lateral to the mandibular ramus. bone resorption was not found at the site of fixation . The left cheek and mandibular body were slightly depressed and a sensory deficit existed in the lower lip. However. mouth opening had increased to about 40 mm. The displacement
of the mandible on the side of the lesion was slight. Because teeth still remained on the unaffected side. relatively good occlusal function could be restored by treatment of the _remaining teeth after surgery. Although no denture was fitted to the affected side, oral function was little disturbed. the patient's daily routine could be achieved, and she was quite satisfied with the results (Fig 7).
FIGURE 6. Photomicrographs showing (8) plexiform ameloblastoma (hematoxylin-eosin, original magnification x200) and (A) formation of large stromal cysts (hematoxylin-eosin, original magnification x40).
FIGURE 7. Relatively symmetrical facial appearance at 18 months after surgery
767
NAKASATO ET AL
Discussion The introduction of the panoramic radiograph in routine dental practice has contributed to the earlier detection and treatment of ameloblastoma. However, if a lesion is left unattended for a long time because of a patient's fear of invasive surgery, it can grow remarkably large. This can be followed by the leakage of cystic fluid from the lesion and may result in the development of secondary hypoproteinemia, as found in this case. Four cases of ameloblastoma associated with hypoproteinemia have been reported.l? The histologic diagnosis in these four cases were either follicular 'S or reticular':" type of ameloblastoma. Large ameloblastomas can cause occlusal abnormality and difficulty with food ingestion.P? Patients also may be at risk for hemorrhage due to ulceration and can develop symptoms of anemia. J Furthermore, the general condition of the patient can deteriorate and life may be endangered by pulmonary edema.' The hypoproteinemia can sometimes be considerably improved by administration of protein and albumin preparations, and tissue coagulants':" can be used for hemorrhage, but this does not substitute for excision of the tumor.':" Moreover, the patient described in this article almost totally failed to respond to medication due to the massive leakage of intracystic fluid. The wall of an odontogenic cyst permits proteins
to pcrmcate.P'? Because the molecular weight of albumin is less than that of globulin, it probably passes through the membrane and migrates more easily into the cystic cavity." As a mechanism for the development of hypoproteinemia in gigantic cystic ameloblastoma, two factors are presumed to be involved: the cyst wall acting as a semipermeable membrane and the leakage of intracystic fluid directly through an opening formed in the cyst walI. 3 •5 In this case, the fluid that leaked through the ulcer in the cyst wall flowed so profusely that it was possible to continuously aspirate it using a bougie, thereby causing the hypoproteinemia.
References I. Masuyarna Y, Shiota K: Extensive ameloblastoma of the mandible. Jpn J Oral Surg 2:4, 1956 2. Petriella VI\I: Gigantic ameloblastoma of the mandible. J Oral Surg 32:44, 1974 3. Kubo K, Yasuhiro H, Oka 1\1, et al: Ameloblastoma of the mand ible with hypoproteinemia . Jpn J Oral Surg 23:291, 1977 4. Osaki T, Ryoke T: Amelobl astoma with hypoproteinemia due to protein leakage. Int J Oral Surg 14:302, 1985 5. Yoshikawa F, Higuchi K, Tsujino M, et al: Mandibular ameloblastoma urnplicating severe hypoproteinemia: Report of a case. Jpn J Oral Maxillofac Surg 36:589, 1990 6. Toller PA: Protein sub stances in odontogenic cyst fluid. Br Dent J 128:317, 1970 7. Kaneko I: Biochemic al studies on cystic fluid of the jaw. Jpn J Oral Surg 18:2, 1972