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Sarcomatous overgrowth in recurrent ameloblastic fibrosarcoma
ORAL ONCOLOGY
Oral Oncology 35 (1999) 346±348
Case Report
Sarcomatous overgrowth in recurrent ameloblastic ®brosarcoma Y. Hayashi a,*, I. Tohnai a, M. Ueda a, T. Nagasaka b a Department of Oral Surgery, Nagoya University School of Medicine, 65 Tsuruma-cho, Showa-ku, Nagoya, 466, Japan Division of Pathology, Clinical Laboratory, Nagoya University Hospital, 65 Tsuruma-cho, Showa-ku, Nagoya, 466, Japan
b
Received 28 October 1998; accepted 16 November 1998
Abstract A case of recurrent ameloblastic ®brosarcoma (AFS) in the mandibular molar region of a 22-year-old male is reported. The tumor was ®rst diagnosed as a sarcoma of undetermined origin, because the benign epithelial component of AFS had disappeared after repeated surgical procedures. The lesion grew rapidly in the time from hospital admission until operation, suggesting progression from low to high malignancy. The correlation between the benign epithelial component and malignancy is discussed. # 1999 Elsevier Science Ltd. All rights reserved. Keywords: Ameloblastic ®brosarcoma; Ameloblastic ®broma; Ameloblastoma
1. Introduction Ameloblastic ®brosarcoma (AFS) is an extremely rare odontogenic malignant neoplasm. In recent reports, it has been suggested that ameloblastic dentinosarcoma and ameloblastic odontosarcoma are the same as AFS, because of the unvarying biological behavior of these neoplasms [1±5]. AFS is composed of a benign epithelial component and malignant mesenchymal tissue. It has been reported that the epithelial component of AFS eventually becomes less prominent and may disappear altogether after local recurrences [6±8]. This paper presents an additional case of AFS and discusses the correlation between the epithelial component and malignancy. 2. Case report A 22-year-old male patient was referred to the Department of Oral Surgery at Nagoya University Hospital, Nagoya, Japan, on 24 January 1995 for evaluation of a tender swelling of the right buccal region and the loss of feeling in the lower lip. The patient ®rst noticed * Corresponding author. Tel.: +81-52-744-2348; Fax: +81-52-7442352; E-mail: [email protected]
swelling of the right side of the mandible in July 1988. He visited a local dental clinic, where X-ray examination indicated a lesion in the right mandible which included the unerupted second molar. He was then referred to a city hospital, where he was diagnosed by biopsy as having ameloblastoma. Enucleation of the tumor was performed on 16 August 1988. He later underwent reoperations in January 1989, September 1991 and June 1994 due to repeated recurrences. The ®ndings at our hospital from a general physical examination were normal and laboratory values were within normal limits. Clinically, his face was asymmetrical with enlargement of the buccal region. Intraoral examination revealed a diuse swelling from the lower buccal posterior gingiva to the soft tissue of the cheek, and a rough, shallow pit which bled easily (Fig. 1). A panoramic radiograph of the region showed bone resorption from the right premolar to the ramus region. A CT scan revealed a mass lesion of 3.5 cm in diameter, which was enhanced heterogeneously and had a low density area inside (Fig. 2a). A T2-weighted MR image clearly showed high-intensity signals in the lesion (Fig. 2b). An incisional biopsy was taken from the buccal side of the lower gingiva at the molar site, and microscopic examination showed scattered or fascicular proliferation of atypical cells exhibiting marked pleomorphism,
1368-8375/99/$ - see front matter # 1999 Elsevier Science Ltd. All rights reserved. PII: S1368 -8 375(98)00123 -7
Y. Hayashi et al./Oral Oncology 35 (1999) 346±348
hyperchromasia and numerous mitoses (Fig. 3). The biopsy diagnosis was of a sarcoma of undetermined origin. We then obtained the primary biopsy specimen which had been diagnosed as ameloblastoma at a previous hospital for review. We con®rmed that in this specimen pleomorphic ®broblasts were surrounding an island of ameloblastic epithelium, and diagnosed AFS (Fig. 4). During the period from admission to operation the tumor grew rapidly (Fig. 5). An extensive excision on 1 March 1995 included hemimandibulectomy and partial maxillectomy. Complex reconstruction of the surgical defect was performed using the rectus abdominis free ¯ap, the ®bula free ¯ap and dental titanium implants. Three years after surgery, clinical and radiographic follow-up of the patient revealed no recurrence of the tumor.
Fig. 1. Intraoral view on admission to hospital.
347
3. Discussion AFS is an extremely rare malignant odontogenic neoplasm. The last review of this disease was by Muller et al. [5], who identi®ed a total of 51 cases. Since that time, ®ve cases have been reported [9±13], making a total of 57 reported cases of AFS, including the one presented here. The average age of presentation for reported cases was 26.8 years, with a range of 3±83 years. It appears more often in the mandible than in the maxilla (3.3:1), and in males than females (1.4:1). AFS has been documented as arising in the jaws either de novo or from a pre-existing benign odontogenic neoplasm. There are many reports of ameloblastic ®broma transforming into AFS after aggressive surgical treatment. The lesion presented in this report may have
Fig. 3. Histological appearance of the biopsy in our department (February 1995). Marked pleomorphism, hyperchromasia and numerous mitoses are seen in the sarcoma tissue [hematoxylin and eosin (H&E)200].
Fig. 2. (a) Computed tomography scan showing the lesion of 3.5 cm in diameter, which was enhanced heterogeneously and had a low density area inside. (b) A T2-weighted magnetic resonance image clearly demonstrating high intensity signals in this lesion.
348
Y. Hayashi et al./Oral Oncology 35 (1999) 346±348
behaves similarly to a low-grade ®brosarcoma or even as a semi-malignant lesion, and may therefore be more appropriately termed proliferating ameloblastic ®broma. However, there is one reported case with histologic documentation of metastases that involved the lung, liver and mediastinal lymph nodes, and loss of the epithelial component [8]. Accordingly the loss of benign odontogenic epithelium is correlated with the malignancy of AFS. We therefore suggest that AFS with total or partial lose of the epithelial component be considered a highly malignant lesion which should be treated as sarcoma. Fig. 4. Histological appearance of the biopsy at the previous hospital (August 1988). The pleomorphic ®broblasts are surrounding an island of ameloblastic epithelium (H&E100).
Fig. 5. Intraoral view just before operation (1 month later than the image in Fig. 1).
arisen de novo as APS, because the patient had not been treated before undergoing biopsy at the previous hospital. It has been reported that the epithelial component of AFS eventually becomes less prominent and may disappear altogether after local recurrences. Park et al. [9] reported a case of highly malignant AFS which showed that sheets of more anaplastic and poorly dierentiated cells were not associated with the benign odontogenic epithelium, whereas less anaplastic mesenchymal tissue was closely associated with it. They suggested that the anaplasia of mesenchymal tissue is correlated with the degeneration of benign odontogenic epithelium, and that the loss of benign odontogenic epithelium results from overgrowth of the malignant mesenchymal portion of the lesion. In the present case, the lesion rapidly grew in size during the time from admission until surgery. Because few reports described lesional lymph node metastases and distant metastases with AFS, most investigators have suggested that clinically AFS
References [1] Adekeye EO, Edward MB, Goubran GF. Transformation of ameloblastic ®broma to ®brosarcoma: report of a case. International Journal of Oral Surgery 1978;7:503±7. [2] Altini M, Thompson SH, Lownie JF, Berezowski BB. Ameloblastic sarcoma of the mandible. Journal of Oral and Maxillofacial Surgery 1985;43:789±94. [3] Wood RM, Mazkle TL, Barker BF, Hiatt WR. Ameloblastic ®brosarcoma. Oral Surgery, Oral Medicine and Oral Pathology 1988;66:74±7. [4] Takeda Y, Kuroda M, Suzui A. Ameloblastic odontosarcoma (Ameloblastic ®bro-odontosarcoma) in the mandible. Acta Pathologica Japonica 1990;40:832±7. [5] Muller S, Parker DC, Kapadia SB, Budnick SD, Barnes EL. Ameloblastic ®brosarcoma of the jaws. A clinicopathologic and DNA analysis of ®ve cases and review of the literature with discussion of its relationship to ameloblastic ®broma. Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology and Endodontics 1995;79:469±77. [6] Leider AS, Nelson JF, Trodahl JN. Ameloblastic ®brosarcoma of the jaws. Oral Surgery, Oral Medicine and Oral Pathology 1972;33:559±69. [7] Goldstein G, Parker FP, Hugh GSF. Ameloblastic sarcoma: pathogenesis and treatment with chemotherapy. Cancer 1976; 37:1673±8. [8] Chomette G, Auriol M, Guilbert F, Delcourt A. Ameloblastic ®brosarcoma of the jaws report of three cases. Pathology, Research and Practice 1983;178:40±7. [9] Park HR, Shin KB, Sol MY, Suh KS, Lee SK. A highly malignant ameloblastic ®brosarcoma. Report of a case. Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology and Endodontics 1995;79:478±81. [10] Takano M, Takizawa T, Kouda H. A case of ameloblastic ®brosarcoma in the molar region of the mandible. Japanese Journal of Oral and Maxillofacial Surgery 1996;42:1203±6. [11] Tajima Y, Utsumi N, Suzuki S, Fujita K. Case report. Ameloblastic ®brosarcoma arising de novo in the maxilla. Pathology International 1997;47:564±8. [12] Nogueira T de O, Carvalho YR, Rosa EB, Dos Santos LM. Possible malignant transformation of an ameloblastic ®broma to ameloblastic ®brosarcoma: a case report. Journal of Oral Maxillo-facial Surgery 1997;55:180±2. [13] Sano K, Yoshida S, Ninomiya H, et al. Assessment of growth potential by MIB-1 immunohistochemistry in ameloblastic ®broma and related lesions of the jaws compared with ameloblastic ®brosarcoma. Journal of Oral Pathology and Medicine 1998;27:59±63.
Oral Oncology 35 (1999) 346±348
Case Report
Sarcomatous overgrowth in recurrent ameloblastic ®brosarcoma Y. Hayashi a,*, I. Tohnai a, M. Ueda a, T. Nagasaka b a Department of Oral Surgery, Nagoya University School of Medicine, 65 Tsuruma-cho, Showa-ku, Nagoya, 466, Japan Division of Pathology, Clinical Laboratory, Nagoya University Hospital, 65 Tsuruma-cho, Showa-ku, Nagoya, 466, Japan
b
Received 28 October 1998; accepted 16 November 1998
Abstract A case of recurrent ameloblastic ®brosarcoma (AFS) in the mandibular molar region of a 22-year-old male is reported. The tumor was ®rst diagnosed as a sarcoma of undetermined origin, because the benign epithelial component of AFS had disappeared after repeated surgical procedures. The lesion grew rapidly in the time from hospital admission until operation, suggesting progression from low to high malignancy. The correlation between the benign epithelial component and malignancy is discussed. # 1999 Elsevier Science Ltd. All rights reserved. Keywords: Ameloblastic ®brosarcoma; Ameloblastic ®broma; Ameloblastoma
1. Introduction Ameloblastic ®brosarcoma (AFS) is an extremely rare odontogenic malignant neoplasm. In recent reports, it has been suggested that ameloblastic dentinosarcoma and ameloblastic odontosarcoma are the same as AFS, because of the unvarying biological behavior of these neoplasms [1±5]. AFS is composed of a benign epithelial component and malignant mesenchymal tissue. It has been reported that the epithelial component of AFS eventually becomes less prominent and may disappear altogether after local recurrences [6±8]. This paper presents an additional case of AFS and discusses the correlation between the epithelial component and malignancy. 2. Case report A 22-year-old male patient was referred to the Department of Oral Surgery at Nagoya University Hospital, Nagoya, Japan, on 24 January 1995 for evaluation of a tender swelling of the right buccal region and the loss of feeling in the lower lip. The patient ®rst noticed * Corresponding author. Tel.: +81-52-744-2348; Fax: +81-52-7442352; E-mail: [email protected]
swelling of the right side of the mandible in July 1988. He visited a local dental clinic, where X-ray examination indicated a lesion in the right mandible which included the unerupted second molar. He was then referred to a city hospital, where he was diagnosed by biopsy as having ameloblastoma. Enucleation of the tumor was performed on 16 August 1988. He later underwent reoperations in January 1989, September 1991 and June 1994 due to repeated recurrences. The ®ndings at our hospital from a general physical examination were normal and laboratory values were within normal limits. Clinically, his face was asymmetrical with enlargement of the buccal region. Intraoral examination revealed a diuse swelling from the lower buccal posterior gingiva to the soft tissue of the cheek, and a rough, shallow pit which bled easily (Fig. 1). A panoramic radiograph of the region showed bone resorption from the right premolar to the ramus region. A CT scan revealed a mass lesion of 3.5 cm in diameter, which was enhanced heterogeneously and had a low density area inside (Fig. 2a). A T2-weighted MR image clearly showed high-intensity signals in the lesion (Fig. 2b). An incisional biopsy was taken from the buccal side of the lower gingiva at the molar site, and microscopic examination showed scattered or fascicular proliferation of atypical cells exhibiting marked pleomorphism,
1368-8375/99/$ - see front matter # 1999 Elsevier Science Ltd. All rights reserved. PII: S1368 -8 375(98)00123 -7
Y. Hayashi et al./Oral Oncology 35 (1999) 346±348
hyperchromasia and numerous mitoses (Fig. 3). The biopsy diagnosis was of a sarcoma of undetermined origin. We then obtained the primary biopsy specimen which had been diagnosed as ameloblastoma at a previous hospital for review. We con®rmed that in this specimen pleomorphic ®broblasts were surrounding an island of ameloblastic epithelium, and diagnosed AFS (Fig. 4). During the period from admission to operation the tumor grew rapidly (Fig. 5). An extensive excision on 1 March 1995 included hemimandibulectomy and partial maxillectomy. Complex reconstruction of the surgical defect was performed using the rectus abdominis free ¯ap, the ®bula free ¯ap and dental titanium implants. Three years after surgery, clinical and radiographic follow-up of the patient revealed no recurrence of the tumor.
Fig. 1. Intraoral view on admission to hospital.
347
3. Discussion AFS is an extremely rare malignant odontogenic neoplasm. The last review of this disease was by Muller et al. [5], who identi®ed a total of 51 cases. Since that time, ®ve cases have been reported [9±13], making a total of 57 reported cases of AFS, including the one presented here. The average age of presentation for reported cases was 26.8 years, with a range of 3±83 years. It appears more often in the mandible than in the maxilla (3.3:1), and in males than females (1.4:1). AFS has been documented as arising in the jaws either de novo or from a pre-existing benign odontogenic neoplasm. There are many reports of ameloblastic ®broma transforming into AFS after aggressive surgical treatment. The lesion presented in this report may have
Fig. 3. Histological appearance of the biopsy in our department (February 1995). Marked pleomorphism, hyperchromasia and numerous mitoses are seen in the sarcoma tissue [hematoxylin and eosin (H&E)200].
Fig. 2. (a) Computed tomography scan showing the lesion of 3.5 cm in diameter, which was enhanced heterogeneously and had a low density area inside. (b) A T2-weighted magnetic resonance image clearly demonstrating high intensity signals in this lesion.
348
Y. Hayashi et al./Oral Oncology 35 (1999) 346±348
behaves similarly to a low-grade ®brosarcoma or even as a semi-malignant lesion, and may therefore be more appropriately termed proliferating ameloblastic ®broma. However, there is one reported case with histologic documentation of metastases that involved the lung, liver and mediastinal lymph nodes, and loss of the epithelial component [8]. Accordingly the loss of benign odontogenic epithelium is correlated with the malignancy of AFS. We therefore suggest that AFS with total or partial lose of the epithelial component be considered a highly malignant lesion which should be treated as sarcoma. Fig. 4. Histological appearance of the biopsy at the previous hospital (August 1988). The pleomorphic ®broblasts are surrounding an island of ameloblastic epithelium (H&E100).
Fig. 5. Intraoral view just before operation (1 month later than the image in Fig. 1).
arisen de novo as APS, because the patient had not been treated before undergoing biopsy at the previous hospital. It has been reported that the epithelial component of AFS eventually becomes less prominent and may disappear altogether after local recurrences. Park et al. [9] reported a case of highly malignant AFS which showed that sheets of more anaplastic and poorly dierentiated cells were not associated with the benign odontogenic epithelium, whereas less anaplastic mesenchymal tissue was closely associated with it. They suggested that the anaplasia of mesenchymal tissue is correlated with the degeneration of benign odontogenic epithelium, and that the loss of benign odontogenic epithelium results from overgrowth of the malignant mesenchymal portion of the lesion. In the present case, the lesion rapidly grew in size during the time from admission until surgery. Because few reports described lesional lymph node metastases and distant metastases with AFS, most investigators have suggested that clinically AFS
References [1] Adekeye EO, Edward MB, Goubran GF. Transformation of ameloblastic ®broma to ®brosarcoma: report of a case. International Journal of Oral Surgery 1978;7:503±7. [2] Altini M, Thompson SH, Lownie JF, Berezowski BB. Ameloblastic sarcoma of the mandible. Journal of Oral and Maxillofacial Surgery 1985;43:789±94. [3] Wood RM, Mazkle TL, Barker BF, Hiatt WR. Ameloblastic ®brosarcoma. Oral Surgery, Oral Medicine and Oral Pathology 1988;66:74±7. [4] Takeda Y, Kuroda M, Suzui A. Ameloblastic odontosarcoma (Ameloblastic ®bro-odontosarcoma) in the mandible. Acta Pathologica Japonica 1990;40:832±7. [5] Muller S, Parker DC, Kapadia SB, Budnick SD, Barnes EL. Ameloblastic ®brosarcoma of the jaws. A clinicopathologic and DNA analysis of ®ve cases and review of the literature with discussion of its relationship to ameloblastic ®broma. Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology and Endodontics 1995;79:469±77. [6] Leider AS, Nelson JF, Trodahl JN. Ameloblastic ®brosarcoma of the jaws. Oral Surgery, Oral Medicine and Oral Pathology 1972;33:559±69. [7] Goldstein G, Parker FP, Hugh GSF. Ameloblastic sarcoma: pathogenesis and treatment with chemotherapy. Cancer 1976; 37:1673±8. [8] Chomette G, Auriol M, Guilbert F, Delcourt A. Ameloblastic ®brosarcoma of the jaws report of three cases. Pathology, Research and Practice 1983;178:40±7. [9] Park HR, Shin KB, Sol MY, Suh KS, Lee SK. A highly malignant ameloblastic ®brosarcoma. Report of a case. Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology and Endodontics 1995;79:478±81. [10] Takano M, Takizawa T, Kouda H. A case of ameloblastic ®brosarcoma in the molar region of the mandible. Japanese Journal of Oral and Maxillofacial Surgery 1996;42:1203±6. [11] Tajima Y, Utsumi N, Suzuki S, Fujita K. Case report. Ameloblastic ®brosarcoma arising de novo in the maxilla. Pathology International 1997;47:564±8. [12] Nogueira T de O, Carvalho YR, Rosa EB, Dos Santos LM. Possible malignant transformation of an ameloblastic ®broma to ameloblastic ®brosarcoma: a case report. Journal of Oral Maxillo-facial Surgery 1997;55:180±2. [13] Sano K, Yoshida S, Ninomiya H, et al. Assessment of growth potential by MIB-1 immunohistochemistry in ameloblastic ®broma and related lesions of the jaws compared with ameloblastic ®brosarcoma. Journal of Oral Pathology and Medicine 1998;27:59±63.