Clinical Review & Education
Clinical Challenge | PATHOLOGY
Ethmoid Sinus Mass Zao Yang, MD; Ravindra Uppaluri, MD, PhD; James S. Lewis Jr, MD
A
B
C
D
Figure. Ethmoid sinus mass. A, Computed tomographic scan. The arrowhead indicates the mass. B, Blastema as indicated by the asterisk and malignant glands as indicated by the arrowhead. C, Rhabdomyoblasts as indicated by the arrowheads (hematoxylin-eosin, original magnification ×40). D, Desmin immunohistochemistry with strong positive staining in the rhabdomyoblasts (original magnification ×20).
A man in his 50s presented with a 7-month history of worsening, primarily left-sided, sinus obstruction and congestion with associated hyposmia, facial pressure, and headaches. He had no history of sinus disease or surgery and had no epistaxis, weight loss, or visual symptoms. Computed tomography showed a 3.1-cm mass in the left ethmoid sinus and nasal cavity (Figure, A). Positron emission tomographic scanning Quiz at jamaotolaryngology.com showed no evidence of metastasis. Nasal endoscopy revealed a friable, polypoid mass in the ethmoid sinus with extension into the nasal cavity and superiorly to the skull base. A biopsy specimen showed a heterogeneous tumor with small, angulated, blue cells with hyperchromatic nuclei, apoptosis, and mitosis, malignant glands lined by cuboidal cells with round nuclei, prominent nucleoli, and eosinophilic cytoplasm. The glands had luminal mucin production (Figure, B). Focally, there were also scattered round cells with abundant eosinophilic, refractile cytoplasm, features of rhabdomyoblasts (Figure, C). These latter cells were strongly positive for desmin (Figure, D) and myogenin.
jamaotolaryngology.com
WHAT IS YOUR DIAGNOSIS?
A. Olfactory neuroblastoma B. Sinonasal teratocarcinosarcoma C. Small cell carcinoma D. Alveolar rhabdomyosarcoma
(Reprinted) JAMA Otolaryngology–Head & Neck Surgery April 2015 Volume 141, Number 4
Copyright 2015 American Medical Association. All rights reserved.
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389
Clinical Review & Education Clinical Challenge
Diagnosis B. Sinonasal teratocarcinosarcoma
Discussion Sinonasal teratocarcinosarcoma (SNTCS) is a rare and aggressive malignant neoplasm with fewer than 100 reported cases. It most commonly involves the nasal cavity and paranasal sinuses, particularly the ethmoid and maxillary sinuses, although cases have been reported in the oral cavity, nasopharynx, and orbit.1 It occurs primarily in men at a mean age of 55 to 60 years.1,2 The clinical differential diagnosis for an upper nasal cavity mass includes nonneoplastic, benign, and malignant lesions. The latter includes olfactory neuroblastoma, squamous cell carcinoma, lymphoma, melanoma, neuroendocrine carcinoma, rhabdomyosarcoma, sinonasal undifferentiated carcinoma, and many other rarer entities. SNTCS is so rare as to be very low on this list. SNTCS most commonly presents with nasal obstruction (in 76% of cases) and epistaxis (in 63%). Frontal headaches are relatively common (in 20%), but other symptoms, such as visual changes, proptosis, anosmia or hyposmia, airway obstruction, and frontal loberelated neurologic changes, occur in less than 10% of cases. 1 Tumors are large at presentation, averaging 5.3 cm.1 Histologically, SNTCS has a highly complex and variable mixture of immature neural or blastemal-appearing components and various benign, atypical, or overtly malignant epithelial and mesenchymal elements without features of specific germ cell tumors, such as seminoma, embryonal carcinoma, choriocarcinoma, or yolk sac tumor.1,3-5 The epithelial component is typically immature and comprises “fetal appearing” squamous cells in 50% to 75% of cases.3 Ciliated columnar and mucin-producing cells with glandular and duct formation are also common.5 Mesenchymal elements possible in SNTCS include fibroblasts, cartilage, and smooth or striated muscle.1 Rhabdomyoblasts (primitive skeletal muscle cells) are sometimes seen and consist of polygonal or spindle-shaped cells with refractile, eosinophilic cytoplasm.5 Primitive neuroectodermal or blastemal components are characterized by small,
Author Affiliations: Department of Otolaryngology–Head and Neck Surgery, Louisiana State University Health Sciences Center, Shreveport (Yang); Department of Otolaryngology–Head and Neck Surgery, Washington University School of Medicine, St Louis, Missouri (Uppaluri, Lewis); Department of Pathology and Immunology, Washington University School of Medicine, St Louis, Missouri (Lewis). Corresponding Author: James S. Lewis Jr, MD, Department of Pathology and Immunology, Washington University in St Louis, 660 S Euclid Ave, Campus Box 8118, St Louis, MO 63110 ([email protected]). Published Online: February 19, 2015. doi:10.1001/jamaoto.2015.22.
390
REFERENCES
5. Endo H, Hirose T, Kuwamura KI, Sano T. Case report: sinonasal teratocarcinosarcoma. Pathol Int. 2001;51(2):107-112.
1. Misra P, Husain Q, Svider PF, Sanghvi S, Liu JK, Eloy JA. Management of sinonasal teratocarcinosarcoma: a systematic review. Am J Otolaryngol. 2014;35(1):5-11.
6. Carrizo F, Pineda-Daboin K, Neto AG, Luna MA. Pharyngeal teratocarcinosarcoma: review of the literature and report of two cases. Ann Diagn Pathol. 2006;10(6):339-342.
2. Wei S, Carroll W, Lazenby A, Bell W, Lopez R, Said-Al-Naief N. Sinonasal teratocarcinosarcoma: report of a case with review of literature and treatment outcome. Ann Diagn Pathol. 2008;12(6): 415-425.
7. Salem F, Rosenblum MK, Jhanwar SC, Kancherla P, Ghossein RA, Carlson DL. Teratocarcinosarcoma of the nasal cavity and paranasal sinuses: report of 3 cases with assessment for chromosome 12p status. Hum Pathol. 2008;39(4):605-609.
3. Heffner DK, Hyams VJ. Teratocarcinosarcoma (malignant teratoma?) of the nasal cavity and paranasal sinuses: a clinicopathologic study of 20 cases. Cancer. 1984;53(10):2140-2154.
8. Sharma HS, Abdullah JM, Othman NH, Muhamad M. Teratocarcinosarcoma of the nasal cavity and ethmoid. J Laryngol Otol. 1998;112(7): 682-686.
Conflict of Interest Disclosures: None reported.
ARTICLE INFORMATION
Section Editor: Edward B. Stelow, MD.
hyperchromatic cells that may be organized in nests, sheets, or rosettes, and stain positively for neuroendocrine markers, such as synaptophysin, chromogranin A, and CD99.2 Staining of the epithelial and mesenchymal elements depends on the particular components of a given tumor. Given their histologic heterogeneity and rarity, SNTCS is commonly misdiagnosed, especially when inadequately sampled during biopsy, with an initial identification rate of roughly 50%.1 SNTCS histogenesis is speculative, with most authors agreeing that it likely arises either from immature pluripotent cells sequestered in the sinonasal tract or from totipotent olfactory epithelial cells that can differentiate into the neuroectodermal, mesenchymal, and epithelial components characteristic of SNTCS.3,6 It is unlikely a germ cell tumor given that it lacks true germ cell tumor elements. This is further evidenced by the lack of chromosome 12p amplification in SNTCS, an alteration which is commonly associated with true germ cell tumors.7 Furthermore, these tumors almost always occur in adult men, a pattern inconsistent with germ cell tumors. Treatment of SNTCS is challenging given its aggressive clinical behavior and is centered on surgical resection. In a literature review by Misra et al1 involving 86 cases (the largest and most complete to date), 87.2% of patients with SNTCS underwent surgery. The most common treatment regimen was surgery with adjuvant radiation therapy, the treatment used in 59.5% of all cases. Chemotherapy with or without surgery or radiation was used in only 18.6% of all cases, although some evidence suggests that the use of chemotherapy in addition to surgery and radiation may improve survival and decrease rates of recurrence and metastasis.1 The patient described herein underwent surgery to negative margins, received postoperative radiation, and is currently without evidence of recurrence 6 months postdiagnosis. The prognosis of SNTCS is poor. Survival rates are reported to be 30% to 54% at 3 years and 20% at 5 years,2,8 with a mean survival of 1.7 to 1.9 years.3,6 Recurrence and metastasis rates are high, occurring in 67% of patients treated with surgery alone2 and 45.7% of patients treated with surgery and radiation.1
4. Shanmugaratnam K, Kunaratnam N, Chia KB, Chiang GS, Sinniah R. Teratoid carcinosarcoma of the paranasal sinuses. Pathology. 1983;15(4):413-419.
JAMA Otolaryngology–Head & Neck Surgery April 2015 Volume 141, Number 4 (Reprinted)
Copyright 2015 American Medical Association. All rights reserved.
Downloaded From: http://archotol.jamanetwork.com/ by a J H Quillen College User on 06/11/2015
jamaotolaryngology.com
Clinical Challenge | PATHOLOGY
Ethmoid Sinus Mass Zao Yang, MD; Ravindra Uppaluri, MD, PhD; James S. Lewis Jr, MD
A
B
C
D
Figure. Ethmoid sinus mass. A, Computed tomographic scan. The arrowhead indicates the mass. B, Blastema as indicated by the asterisk and malignant glands as indicated by the arrowhead. C, Rhabdomyoblasts as indicated by the arrowheads (hematoxylin-eosin, original magnification ×40). D, Desmin immunohistochemistry with strong positive staining in the rhabdomyoblasts (original magnification ×20).
A man in his 50s presented with a 7-month history of worsening, primarily left-sided, sinus obstruction and congestion with associated hyposmia, facial pressure, and headaches. He had no history of sinus disease or surgery and had no epistaxis, weight loss, or visual symptoms. Computed tomography showed a 3.1-cm mass in the left ethmoid sinus and nasal cavity (Figure, A). Positron emission tomographic scanning Quiz at jamaotolaryngology.com showed no evidence of metastasis. Nasal endoscopy revealed a friable, polypoid mass in the ethmoid sinus with extension into the nasal cavity and superiorly to the skull base. A biopsy specimen showed a heterogeneous tumor with small, angulated, blue cells with hyperchromatic nuclei, apoptosis, and mitosis, malignant glands lined by cuboidal cells with round nuclei, prominent nucleoli, and eosinophilic cytoplasm. The glands had luminal mucin production (Figure, B). Focally, there were also scattered round cells with abundant eosinophilic, refractile cytoplasm, features of rhabdomyoblasts (Figure, C). These latter cells were strongly positive for desmin (Figure, D) and myogenin.
jamaotolaryngology.com
WHAT IS YOUR DIAGNOSIS?
A. Olfactory neuroblastoma B. Sinonasal teratocarcinosarcoma C. Small cell carcinoma D. Alveolar rhabdomyosarcoma
(Reprinted) JAMA Otolaryngology–Head & Neck Surgery April 2015 Volume 141, Number 4
Copyright 2015 American Medical Association. All rights reserved.
Downloaded From: http://archotol.jamanetwork.com/ by a J H Quillen College User on 06/11/2015
389
Clinical Review & Education Clinical Challenge
Diagnosis B. Sinonasal teratocarcinosarcoma
Discussion Sinonasal teratocarcinosarcoma (SNTCS) is a rare and aggressive malignant neoplasm with fewer than 100 reported cases. It most commonly involves the nasal cavity and paranasal sinuses, particularly the ethmoid and maxillary sinuses, although cases have been reported in the oral cavity, nasopharynx, and orbit.1 It occurs primarily in men at a mean age of 55 to 60 years.1,2 The clinical differential diagnosis for an upper nasal cavity mass includes nonneoplastic, benign, and malignant lesions. The latter includes olfactory neuroblastoma, squamous cell carcinoma, lymphoma, melanoma, neuroendocrine carcinoma, rhabdomyosarcoma, sinonasal undifferentiated carcinoma, and many other rarer entities. SNTCS is so rare as to be very low on this list. SNTCS most commonly presents with nasal obstruction (in 76% of cases) and epistaxis (in 63%). Frontal headaches are relatively common (in 20%), but other symptoms, such as visual changes, proptosis, anosmia or hyposmia, airway obstruction, and frontal loberelated neurologic changes, occur in less than 10% of cases. 1 Tumors are large at presentation, averaging 5.3 cm.1 Histologically, SNTCS has a highly complex and variable mixture of immature neural or blastemal-appearing components and various benign, atypical, or overtly malignant epithelial and mesenchymal elements without features of specific germ cell tumors, such as seminoma, embryonal carcinoma, choriocarcinoma, or yolk sac tumor.1,3-5 The epithelial component is typically immature and comprises “fetal appearing” squamous cells in 50% to 75% of cases.3 Ciliated columnar and mucin-producing cells with glandular and duct formation are also common.5 Mesenchymal elements possible in SNTCS include fibroblasts, cartilage, and smooth or striated muscle.1 Rhabdomyoblasts (primitive skeletal muscle cells) are sometimes seen and consist of polygonal or spindle-shaped cells with refractile, eosinophilic cytoplasm.5 Primitive neuroectodermal or blastemal components are characterized by small,
Author Affiliations: Department of Otolaryngology–Head and Neck Surgery, Louisiana State University Health Sciences Center, Shreveport (Yang); Department of Otolaryngology–Head and Neck Surgery, Washington University School of Medicine, St Louis, Missouri (Uppaluri, Lewis); Department of Pathology and Immunology, Washington University School of Medicine, St Louis, Missouri (Lewis). Corresponding Author: James S. Lewis Jr, MD, Department of Pathology and Immunology, Washington University in St Louis, 660 S Euclid Ave, Campus Box 8118, St Louis, MO 63110 ([email protected]). Published Online: February 19, 2015. doi:10.1001/jamaoto.2015.22.
390
REFERENCES
5. Endo H, Hirose T, Kuwamura KI, Sano T. Case report: sinonasal teratocarcinosarcoma. Pathol Int. 2001;51(2):107-112.
1. Misra P, Husain Q, Svider PF, Sanghvi S, Liu JK, Eloy JA. Management of sinonasal teratocarcinosarcoma: a systematic review. Am J Otolaryngol. 2014;35(1):5-11.
6. Carrizo F, Pineda-Daboin K, Neto AG, Luna MA. Pharyngeal teratocarcinosarcoma: review of the literature and report of two cases. Ann Diagn Pathol. 2006;10(6):339-342.
2. Wei S, Carroll W, Lazenby A, Bell W, Lopez R, Said-Al-Naief N. Sinonasal teratocarcinosarcoma: report of a case with review of literature and treatment outcome. Ann Diagn Pathol. 2008;12(6): 415-425.
7. Salem F, Rosenblum MK, Jhanwar SC, Kancherla P, Ghossein RA, Carlson DL. Teratocarcinosarcoma of the nasal cavity and paranasal sinuses: report of 3 cases with assessment for chromosome 12p status. Hum Pathol. 2008;39(4):605-609.
3. Heffner DK, Hyams VJ. Teratocarcinosarcoma (malignant teratoma?) of the nasal cavity and paranasal sinuses: a clinicopathologic study of 20 cases. Cancer. 1984;53(10):2140-2154.
8. Sharma HS, Abdullah JM, Othman NH, Muhamad M. Teratocarcinosarcoma of the nasal cavity and ethmoid. J Laryngol Otol. 1998;112(7): 682-686.
Conflict of Interest Disclosures: None reported.
ARTICLE INFORMATION
Section Editor: Edward B. Stelow, MD.
hyperchromatic cells that may be organized in nests, sheets, or rosettes, and stain positively for neuroendocrine markers, such as synaptophysin, chromogranin A, and CD99.2 Staining of the epithelial and mesenchymal elements depends on the particular components of a given tumor. Given their histologic heterogeneity and rarity, SNTCS is commonly misdiagnosed, especially when inadequately sampled during biopsy, with an initial identification rate of roughly 50%.1 SNTCS histogenesis is speculative, with most authors agreeing that it likely arises either from immature pluripotent cells sequestered in the sinonasal tract or from totipotent olfactory epithelial cells that can differentiate into the neuroectodermal, mesenchymal, and epithelial components characteristic of SNTCS.3,6 It is unlikely a germ cell tumor given that it lacks true germ cell tumor elements. This is further evidenced by the lack of chromosome 12p amplification in SNTCS, an alteration which is commonly associated with true germ cell tumors.7 Furthermore, these tumors almost always occur in adult men, a pattern inconsistent with germ cell tumors. Treatment of SNTCS is challenging given its aggressive clinical behavior and is centered on surgical resection. In a literature review by Misra et al1 involving 86 cases (the largest and most complete to date), 87.2% of patients with SNTCS underwent surgery. The most common treatment regimen was surgery with adjuvant radiation therapy, the treatment used in 59.5% of all cases. Chemotherapy with or without surgery or radiation was used in only 18.6% of all cases, although some evidence suggests that the use of chemotherapy in addition to surgery and radiation may improve survival and decrease rates of recurrence and metastasis.1 The patient described herein underwent surgery to negative margins, received postoperative radiation, and is currently without evidence of recurrence 6 months postdiagnosis. The prognosis of SNTCS is poor. Survival rates are reported to be 30% to 54% at 3 years and 20% at 5 years,2,8 with a mean survival of 1.7 to 1.9 years.3,6 Recurrence and metastasis rates are high, occurring in 67% of patients treated with surgery alone2 and 45.7% of patients treated with surgery and radiation.1
4. Shanmugaratnam K, Kunaratnam N, Chia KB, Chiang GS, Sinniah R. Teratoid carcinosarcoma of the paranasal sinuses. Pathology. 1983;15(4):413-419.
JAMA Otolaryngology–Head & Neck Surgery April 2015 Volume 141, Number 4 (Reprinted)
Copyright 2015 American Medical Association. All rights reserved.
Downloaded From: http://archotol.jamanetwork.com/ by a J H Quillen College User on 06/11/2015
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