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Figure.
Composite photomicrograph of different phenotypic features of teratocarcinosarcoma (hematoxylin-eosin, original magnification ×40). A, Neural filaments and primitive mesenchymal background; B, primitive neuroepithelium with rosette formation; C, mature and immature rhabdomyoblastic with focal primitive neuroepithelium; and D, epithelial glandular carcinoma (at right) and osteosarcoma component (at left).

Composite photomicrograph of different phenotypic features of teratocarcinosarcoma (hematoxylin-eosin, original magnification ×40). A, Neural filaments and primitive mesenchymal background; B, primitive neuroepithelium with rosette formation; C, mature and immature rhabdomyoblastic with focal primitive neuroepithelium; and D, epithelial glandular carcinoma (at right) and osteosarcoma component (at left).

Table. 
Demographic and Clinical Features of Patients With Sinonasal Teratocarcinosarcoma
Demographic and Clinical Features of Patients With Sinonasal Teratocarcinosarcoma
1.
Shanmugaratnam  K International Histological Classification of Tumors.  Berlin, Germany Springer-Verlag1991;70- 71
2.
Redondo Martínez  ERey Lopez  AReguera Parra  VBolanos Rodriguez  C Sinusal teratocarcinosarcoma. Acta Otorrinolaringol Esp 1991;42 (5) 363- 367
PubMed
3.
Prasad  KCPai  RRPadmanabhan  KChawla  S Teratocarcinosarcoma of the nose, paranasal sinuses and nasopharynx. J Laryngol Otol 2003;117 (4) 321- 324
PubMedArticle
4.
Shemen  LGalantich  PMurali  R Malignant teratocarcinosarcoma of the sphenoid sinus. Otolaryngol Head Neck Surg 1995;112 (3) 496- 500
PubMedArticle
5.
Fukuoka  KHirokawa  MShimizu  M  et al.  Teratocarcinosarcoma of the nasal cavity: report of a case showing favorable prognosis. APMIS 2000;108 (9) 553- 557
PubMedArticle
6.
Chao  KKEng  TYBarnes  JDahiya  R Sinonasal teratocarcinosarcoma. Am J Clin Oncol 2004;27 (1) 29- 32
PubMedArticle
7.
Szudek  JBullock  MTaylor  SM Sinonasal teratocarcinosarcoma involving the cavernous sinus. J Otolaryngol 2005;34 (4) 286- 288
PubMedArticle
8.
Pai  SANaresh  KNMasih  KRamarao  CBorges  AM Teratocarcinosarcoma of the paranasal sinuses: a clinicopathologic and immunohistochemical study. Hum Pathol 1998;29 (7) 718- 722
PubMedArticle
9.
Kleinschmidt-DeMasters  BKPflaumer  SMulgrew  TLillehei  K Sinonasal teratocarcinosarcoma (“mixed olfactory neuroblastoma-craniopharyngioma”) presenting with syndrome of inappropriate secretion of antidiuretic hormone. Clin Neuropathol 2000;19 (2) 63- 69
PubMed
10.
Endo  HHirose  TKuwamura  KISano  T Case report: sinonasal teratocarcinosarcoma. Pathol Int 2001;51 (2) 107- 112
PubMedArticle
11.
Batsakis  JGEl-Naggar  AKLuna  MA Teratomas of the head and neck with emphasis on malignancy. Ann Otol Rhinol Laryngol 1995;104 (6) 496- 500
PubMed
12.
Heffner  DKHyams  VJ Teratocarcinosarcoma (malignant teratoma?) of the nasal cavity and paranasal sinuses: a clinicopathologic study of 20 cases. Cancer 1984;53 (10) 2140- 2154
PubMedArticle
13.
Terasaka  SMedary  MBWhiting  DMFukushima  TEspejo  EJNathan  G Prolonged survival in a patient with sinonasal teratocarcinosarcoma with cranial extension: case report. J Neurosurg 1998;88 (4) 753- 756
PubMedArticle
14.
Shindo  MLStanley  RB  JrKiyabu  MT Carcinosarcoma of the nasal cavity and paranasal sinuses. Head Neck 1990;12 (6) 516- 519
PubMedArticle
15.
Christensen  EFu  YWilson  LHoover  L Teratoid carcinosarcoma of the nasal and paranasal cavities: a case report. Am J Rhinol 1992;6 (5) 169- 172Article
16.
Devgan  BKDevgan  MGross  CW Teratocarcinoma of the ethmoid sinus: review of literature plus a new case report. Otolaryngology 1978;86 (5) 689- 695
17.
Okulicz-Kozaryn  PGolusinski  JMajewski  C A case of tumour teratoides malignum, originating from the accessory sinuses of the nose. Otolaryngol Pol 1958;12309- 314
18.
Sharma  HSAbdullah  JMOthman  NHMuhamad  M Teratocarcinosarcoma of the nasal cavity and ethmoid. J Laryngol Otol 1998;112 (7) 682- 686
PubMedArticle
19.
Takasaki  KSakihama  NTakahashi  H A case with sinonasal teratocarcinosarcoma in the nasal cavity and ethmoid sinus. Eur Arch Otorhinolaryngol 2006;263 (6) 586- 591
PubMedArticle
20.
Ling  FTGerin-Lajoie  JWang  D Sinonasal teratocarcinosarcoma. J Otolaryngol 2004;33 (3) 203- 206
PubMedArticle
21.
Abt  ABToker  C Malignant teratoma of the paranasal sinuses. Arch Pathol 1970;90 (2) 176- 180
PubMed
22.
Shanmugaratnam  KKunaratnam  NChia  KBChiang  GSSinniah  R Teratoid carcinosarcoma of the paranasal sinuses. Pathology 1983;15 (4) 413- 419
PubMedArticle
23.
Shimazaki  HAida  STamai  SMiyazawa  TNakanobou  M Sinonasal teratocarcinosarcoma: ultrastructural and immunohistochemical evidence of neuroectodermal origin. Ultrastruct Pathol 2000;24 (2) 115- 122
PubMedArticle
24.
Fernández  PLCardesa  AAlos  LPinto  JTraserra  J Sinonasal teratocarcinosarcoma: an unusual neoplasm. Pathol Res Pract 1995;191 (2) 166- 171
PubMedArticle
25.
Ogawa  TIkeda  KWatanabe  M  et al.  A case report of sinonasal teratocarcinosarcoma. Tohoku J Exp Med 2000;190 (1) 51- 59
PubMedArticle
26.
Meinecke  RBauer  FSkouras  JMottu  F Blastomatous tumors of the respiratory tract. Cancer 1976;38 (2) 818- 823
PubMedArticle
27.
Patchefsky  ASundmaker  WMarden  PA Malignant teratoma of the ethmoid sinus: report of a case. Cancer 1968;21 (4) 714- 721
PubMedArticle
28.
Patterson  SDBallard  RW Nasal blastoma: a light and electron microscopic study. Ultrastruct Pathol 1980;1 (4) 487- 494
PubMedArticle
29.
Petrovich  ZWollman  JAcquarelli  MBarton  R Malignant teratoma of the nasal cavity. J Surg Oncol 1977;9 (1) 21- 28
PubMedArticle
30.
Dicke  TEGates  GA Malignant teratoma of the paranasal sinuses: report of a case. Arch Otolaryngol 1970;91 (4) 391- 394
PubMedArticle
31.
Nitsche  MHermann  RMChristiansen  HBerger  JPradier  O Rationale for individualized therapy in sinonasal teratocarcinosarcoma (SNTC): case report. Onkologie 2005;28 (12) 653- 656
PubMedArticle
Original Article
June 01, 2008

Sinonasal Teratocarcinosarcoma of the Head and NeckA Report of 10 Patients Treated at a Single Institution and Comparison With Reported Series

Author Affiliations

Author Affiliations: Department of Head and Neck Surgery, The University of Texas Medical School (Dr Smith), and the Departments of Head and Neck Surgery (Drs Hessel and El-Naggar), Pathology (Drs Luna, Malpica, and El-Naggar), and Radiation Oncology (Dr Rosenthal), The University of Texas M. D. Anderson Cancer Center, Houston.

Arch Otolaryngol Head Neck Surg. 2008;134(6):592-595. doi:10.1001/archotol.134.6.592
Abstract

Objectives  To present the clinicopathologic features of 10 sinonasal teratocarcinosarcomas managed at a single center. Teratocarcinosarcoma is a rare morphologically heterogeneous and highly malignant neoplasm. Previous reports of these tumors have focused on their differential diagnosis and histogenesis and consisted of individual case reports and consultation based series.

Design  Review of patient medical records and microscopic slides of all tumor tissues. The histopathologic features for each tumor and the demographic, clinical, treatment, and follow-up information were recorded for each patient. Also, a comparison with previously reported series was performed.

Setting  The University of Texas M. D. Anderson Cancer Center.

Patients  Ten men ranging in age from 35 to 69 years (mean age, 53 years) were included in the study. They all experienced a short course of symptoms, with an average duration of 3.5 months, and 9 presented with nasal obstruction and epistaxis. Nine patients were treated with both surgery and irradiation.

Results  Histologically, the tumors showed primitive neuroepithelial elements and various malignant epithelial and mesenchymal components. Six patients had no evidence of disease by the end of follow-up, which ranged from 72 to 372 months. Three patients died of disease, and 1 patient was lost to follow-up.

Conclusion  Sinonasal teratocarcinosarcoma of the head and neck is a histologically and biologically heterogeneous malignant neoplasm that is best managed with surgery and postoperative radiotherapy.

Sinonasal teratocarcinosarcoma is an extremely rare malignant neoplasm of uncertain histogenesis.1,2 Because of the infrequency and the complex phenotypic composition, these lesions are often misdiagnosed, leading to management difficulties.310 The origin of these tumors remains undetermined, although a stem or pluripotential progenitor cell with multidirectional differentiation is a likely candidate.11 Phenotypically, these tumors are composed of benign neural elements and various malignant epithelial and mesenchymal components.12 The carcinoma may be either squamous or adenocarcinoma, and the mesenchymal component may manifest spindle, smooth, skeletal muscle, cartilage, and bone features.

Reports of the clinical findings of these tumors differ widely in patient presentations, management techniques, and biological outcomes.514 This wide variation is largely related to the nature of the publications: single case reports and consultative series. Furthermore, the objectives of the vast majority of these studies centered on the pathomorphology, differential diagnosis, and histogenesis, with less emphasis on the clinical behavior and treatment outcome.1523 The clinical features and treatment of patients with these tumors, therefore, remained to be addressed.24 In the present study, to our knowledge the largest from a single institution, we present our experience with 10 sinonasal teratocarcinosarcoma cases and a review of the literature.

METHODS
PATIENTS AND TUMORS

Ten sinonasal teratocarcinosarcomas were identified through an extensive review of sinonasal tumors at The University of Texas M. D. Anderson Cancer Center, Department of Pathology, between 1965 and 2000. The clinicopathologic, treatment, and follow-up information is summarized in the Table. All 10 patients were men who ranged in age from 35 to 69 years (average age, 53 years). Nine of the 10 presented with nasal obstruction and epistaxis, and 1 with only epistaxis. The course of their symptoms was fairly short, with durations ranging from 1 to 8 months (average duration, 3.5 months). The initial diagnoses of these cases included poorly differentiated malignant neoplasm, poorly differentiated squamous carcinoma, malignant mixed mesodermal tumor, spindle cell squamous carcinoma, teratocarcinosarcoma, and neuroendocrine tumors, among others.

PATHOLOGIC FEATURES

Histologically, all 10 tumors manifested variable phenotypic features representing the main histogenetic components of this entity. All tumors contained malignant neuroepithelial elements and various epithelial and mesenchymal malignant components (Figure). The dominant malignant phenotypes were adenocarcinoma and squamous carcinoma in 8 cases, rhabdomyosarcoma in 6, and tumor contained within chondrosarcoma areas in 2. In all tumors, however, differing components of neuroepithelial, epithelial, and mesenchymal malignant areas were present.

RADIOGRAPHIC FEATURES

Computed tomography images were reviewed and established that 9 of the tumors were localized to the nasal cavity. The location was left sided in 6 and right sided in 4 of the patients. Five patients had tumor extending into the maxillary sinus; 6 patients had extension into the ethmoid sinus; and 2 had extension into the sphenoid sinus. One patient had a mass located only in the paranasal sinuses with no mass in the nasal cavity.

RESULTS

All patients but 1 underwent surgery as the initial treatment, and this included 4 maxillectomies, 2 sphenoidectomies, 3 ethmoidectomies, and 1 orbital exenteration. Of the 9 patients who underwent postoperative radiotherapy, information on the dose and the rate of radiation administered was available for 7 patients. Five of these were given 60 Gy in 30 fractions; 1 received 54 Gy in 27 fractions; and 1 received 25 Gy in 5 fractions. Radiation therapy was directed to the primary site only, and no patient received elective nodal irradiation. A variety of megavoltage beam types, energies, and techniques were used over the long interval of this review. Only 1 patient was preoperatively treated with chemotherapy and died 1 day postoperatively of myocardial infarction. Eight patients had adequate follow-up, and 1 patient was lost to follow-up. Of the 8 patients with follow-up information, 3 died of locoregional failure at an average of 15 months after treatment, and 5 had no evidence of disease after long follow-up (average follow-up, 200 months).

COMMENT

This study, to our knowledge the largest series performed at a single institution, presents the salient pathologic and clinical characteristics and the treatment outcomes of patients with these rare tumors. All patients were men, with an average age of 53 years. They most frequently presented with a brief history of epistaxis and nasal obstruction. Rare symptoms included eye and/or facial pain, expectoration of tissue, epiphora, headache, vision loss, exophthalmos, and anosmia. One of our patients presented with syndrome of inappropriate antidiuretic hormone secretion and a headache, while another presented with headache, somnolence, and apathy. In previously reported series, most of the patients were also male; only 11% were female with similar symptoms.215 The duration of symptoms in our patients averaged 3.5 months and was comparable to that reported in most of the reported studies.521 The short onset in these cases is most likely due to the rapid growth and the extensive local destruction by this entity compared with other tumors of the sinonasal tract.3,14,25

Most of the tumors in our series, as in previous reports, showed extension to adjacent structures at the time of presentation, with the nasal cavity and/or the paranasal sinuses being the most frequently affected. In some cases, the site origin could not be determined, most likely owing to the rapid growth and the involvement of adjacent structures. Restricted involvement of the paranasal sinuses was observed in a small number of cases. In decreasing order of frequency, tumors were found in the orbit, skull base, cribriform plate, nasopharynx, and anterior cranial fossa.1620

Histopathologically, most of our patients presented with different diagnoses of diverse histogenesis that included different epithelial, germ cell, and mesenchymal malignant neoplasms. This diversity of diagnoses is mainly related to unfamiliarity and the marked phenotypic heterogeneity of these tumors.10 Accurate diagnosis requires adequate diagnostic specimens and experience with this entity.11,12,20,21,2530 Features that may aid in the recognition of these tumors are the presence of immature neural tissue and fetal epithelial structure admixed with various malignant and mesenchymal elements.11,12,2124 Although the histogenesis of these tumors remains a subject of debate, an origin from a primitive neural crest or stem or progenitor cell with multidirectional differentiation is currently favored.9,11

The management of all of our cases was generally similar: 9 of our patients underwent surgery and postoperative radiotherapy. Of these, 5 survived with no evidence of recurrence; 1 was lost to follow-up; and 3 died of their disease due to locoregional failure. These findings, along with those of previously reported studies, show a recurrence rate of approximately 37% and a mortality rate of 30%.16,24 In previous series, however, patients who were treated with surgery alone had a high recurrence rate and moderate mortality rate in contrast to those receiving radiation treatment.16,24 Only 1 of our patients, who received chemotherapy and radiotherapy, developed cervical metastasis and died of disease.

In summary, sinonasal teratocarcinosarcoma is a rare tumor characterized by the presence of benign and malignant epithelial, mesenchymal, and neural components. Owing to its heterogeneous histologic appearance, adequate sampling and recognition of all the components of this type of tumor are needed for its correct diagnosis. Sinonasal teratocarcinosarcoma has been reported to be a highly aggressive tumor. However, the long survival seen in some patients underscores the importance of tumor staging and assessing resectability in patients with sinonasal teratocarcinosarcoma. Surgical removal, as complete as possible, and postoperative adjuvant therapy seem to be the treatments of choice for patients with sinonasal teratocarcinosarcoma. Future individualized therapy may also hold promise.31

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Article Information

Correspondence: Adel K. El-Naggar, MD, PhD, Department of Pathology, The University of Texas M. D. Anderson Cancer Center, 1515 Holcombe Blvd, Unit 085, Houston, TX 77030 (anaggar@mdanderson.org).

Submitted for Publication: June 15, 2007; final revision received September 7, 2007; accepted September 11, 2007.

Author Contributions: Dr El-Naggar had full access to all the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis. Study concept and design: Smith, Luna, Malpica, Rosenthal, and El-Naggar. Acquisition of data: Smith, Malpica, and Rosenthal. Analysis and interpretation of data: Smith, Hessel, and Rosenthal. Drafting of the manuscript: Smith. Critical revision of the manuscript for important intellectual content: Smith, Hessel, Luna, Malpica, Rosenthal, and El-Naggar. Administrative, technical, and material support: Smith, Rosenthal, and El-Naggar. Study supervision: Hessel, Luna, Malpica, Rosenthal, and El-Naggar.

Financial Disclosure: None reported.

Funding/Support: This work was supported in part by the Kenneth D. Müller Professorship (Dr El-Naggar).

References
1.
Shanmugaratnam  K International Histological Classification of Tumors.  Berlin, Germany Springer-Verlag1991;70- 71
2.
Redondo Martínez  ERey Lopez  AReguera Parra  VBolanos Rodriguez  C Sinusal teratocarcinosarcoma. Acta Otorrinolaringol Esp 1991;42 (5) 363- 367
PubMed
3.
Prasad  KCPai  RRPadmanabhan  KChawla  S Teratocarcinosarcoma of the nose, paranasal sinuses and nasopharynx. J Laryngol Otol 2003;117 (4) 321- 324
PubMedArticle
4.
Shemen  LGalantich  PMurali  R Malignant teratocarcinosarcoma of the sphenoid sinus. Otolaryngol Head Neck Surg 1995;112 (3) 496- 500
PubMedArticle
5.
Fukuoka  KHirokawa  MShimizu  M  et al.  Teratocarcinosarcoma of the nasal cavity: report of a case showing favorable prognosis. APMIS 2000;108 (9) 553- 557
PubMedArticle
6.
Chao  KKEng  TYBarnes  JDahiya  R Sinonasal teratocarcinosarcoma. Am J Clin Oncol 2004;27 (1) 29- 32
PubMedArticle
7.
Szudek  JBullock  MTaylor  SM Sinonasal teratocarcinosarcoma involving the cavernous sinus. J Otolaryngol 2005;34 (4) 286- 288
PubMedArticle
8.
Pai  SANaresh  KNMasih  KRamarao  CBorges  AM Teratocarcinosarcoma of the paranasal sinuses: a clinicopathologic and immunohistochemical study. Hum Pathol 1998;29 (7) 718- 722
PubMedArticle
9.
Kleinschmidt-DeMasters  BKPflaumer  SMulgrew  TLillehei  K Sinonasal teratocarcinosarcoma (“mixed olfactory neuroblastoma-craniopharyngioma”) presenting with syndrome of inappropriate secretion of antidiuretic hormone. Clin Neuropathol 2000;19 (2) 63- 69
PubMed
10.
Endo  HHirose  TKuwamura  KISano  T Case report: sinonasal teratocarcinosarcoma. Pathol Int 2001;51 (2) 107- 112
PubMedArticle
11.
Batsakis  JGEl-Naggar  AKLuna  MA Teratomas of the head and neck with emphasis on malignancy. Ann Otol Rhinol Laryngol 1995;104 (6) 496- 500
PubMed
12.
Heffner  DKHyams  VJ Teratocarcinosarcoma (malignant teratoma?) of the nasal cavity and paranasal sinuses: a clinicopathologic study of 20 cases. Cancer 1984;53 (10) 2140- 2154
PubMedArticle
13.
Terasaka  SMedary  MBWhiting  DMFukushima  TEspejo  EJNathan  G Prolonged survival in a patient with sinonasal teratocarcinosarcoma with cranial extension: case report. J Neurosurg 1998;88 (4) 753- 756
PubMedArticle
14.
Shindo  MLStanley  RB  JrKiyabu  MT Carcinosarcoma of the nasal cavity and paranasal sinuses. Head Neck 1990;12 (6) 516- 519
PubMedArticle
15.
Christensen  EFu  YWilson  LHoover  L Teratoid carcinosarcoma of the nasal and paranasal cavities: a case report. Am J Rhinol 1992;6 (5) 169- 172Article
16.
Devgan  BKDevgan  MGross  CW Teratocarcinoma of the ethmoid sinus: review of literature plus a new case report. Otolaryngology 1978;86 (5) 689- 695
17.
Okulicz-Kozaryn  PGolusinski  JMajewski  C A case of tumour teratoides malignum, originating from the accessory sinuses of the nose. Otolaryngol Pol 1958;12309- 314
18.
Sharma  HSAbdullah  JMOthman  NHMuhamad  M Teratocarcinosarcoma of the nasal cavity and ethmoid. J Laryngol Otol 1998;112 (7) 682- 686
PubMedArticle
19.
Takasaki  KSakihama  NTakahashi  H A case with sinonasal teratocarcinosarcoma in the nasal cavity and ethmoid sinus. Eur Arch Otorhinolaryngol 2006;263 (6) 586- 591
PubMedArticle
20.
Ling  FTGerin-Lajoie  JWang  D Sinonasal teratocarcinosarcoma. J Otolaryngol 2004;33 (3) 203- 206
PubMedArticle
21.
Abt  ABToker  C Malignant teratoma of the paranasal sinuses. Arch Pathol 1970;90 (2) 176- 180
PubMed
22.
Shanmugaratnam  KKunaratnam  NChia  KBChiang  GSSinniah  R Teratoid carcinosarcoma of the paranasal sinuses. Pathology 1983;15 (4) 413- 419
PubMedArticle
23.
Shimazaki  HAida  STamai  SMiyazawa  TNakanobou  M Sinonasal teratocarcinosarcoma: ultrastructural and immunohistochemical evidence of neuroectodermal origin. Ultrastruct Pathol 2000;24 (2) 115- 122
PubMedArticle
24.
Fernández  PLCardesa  AAlos  LPinto  JTraserra  J Sinonasal teratocarcinosarcoma: an unusual neoplasm. Pathol Res Pract 1995;191 (2) 166- 171
PubMedArticle
25.
Ogawa  TIkeda  KWatanabe  M  et al.  A case report of sinonasal teratocarcinosarcoma. Tohoku J Exp Med 2000;190 (1) 51- 59
PubMedArticle
26.
Meinecke  RBauer  FSkouras  JMottu  F Blastomatous tumors of the respiratory tract. Cancer 1976;38 (2) 818- 823
PubMedArticle
27.
Patchefsky  ASundmaker  WMarden  PA Malignant teratoma of the ethmoid sinus: report of a case. Cancer 1968;21 (4) 714- 721
PubMedArticle
28.
Patterson  SDBallard  RW Nasal blastoma: a light and electron microscopic study. Ultrastruct Pathol 1980;1 (4) 487- 494
PubMedArticle
29.
Petrovich  ZWollman  JAcquarelli  MBarton  R Malignant teratoma of the nasal cavity. J Surg Oncol 1977;9 (1) 21- 28
PubMedArticle
30.
Dicke  TEGates  GA Malignant teratoma of the paranasal sinuses: report of a case. Arch Otolaryngol 1970;91 (4) 391- 394
PubMedArticle
31.
Nitsche  MHermann  RMChristiansen  HBerger  JPradier  O Rationale for individualized therapy in sinonasal teratocarcinosarcoma (SNTC): case report. Onkologie 2005;28 (12) 653- 656
PubMedArticle
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