Neuro-radiology
Neuroradiology (1992) 34:36%369
Head and Neck Radiology
9 Springer-Verlag 1992
Infratemporal alveolar soft part sarcoma: CT, MRI and angiographic findings M. Castillo t , Y. Y. Lee 2, and S. Yamasaki 2
1Sections of Neuroradiology, University of Texas Medical School, L. B.J. General Hospital, and 2University of Texas System Cancer Center, M. D. Anderson Hospital, Houston, Texas, USA Received: 15 April 1991
Summary. The imaging features of a rare alveolar soft part sarcoma found in a 44-year-old female are presented. Although the tumor showed hypervascularity by angiography, CT and MRI suggested slow growth. Despite this relatively benign appearance, alveolar soft part sarcoma is one of the most malignant sarcomas.
gesting vessels, and intense enhancement following the administration of gadolinium-DTPA (Fig.2 a,b). The tumor invaded the dura mater of the right middle cranial fossa (Fig.2 c). At angiography, the hypervascular mass was mainly supplied by an enlarged internal maxillary artery (Fig. 3). It was embolized with Gelfoam and then resected entirely. Pathology examination showed the tumor to be an ASPS.
Key words: Alveolar soft part sarcoma - Soft tissue sarcoma - Sarcoma Discussion
Alveolar soft part sarcoma (ASPS) is an extremely rare tumor accounting for less than 1% of all soft tissue sarcomas. More than half of ASPS occur in the muscles or fascial planes of the lower limbs [1]. We present a case of ASPS presenting in the infratemporal fossa.
Case report
A 44-year-old female presented with a 3-month history of progressive right-sided trismus and pulsatile tinnitus. A year previously she had noted right scalp tenderness which slowly progressed to dysesthesiae of the right side of the face and tongue. Examination revealed diffuse swelling of the right side of the face extending to the preauricular and temporal regions, mild trismus and a mucosa-covered mass in the roof of the right nasopharynx. Contrast-enhanced computed tomography (CT) showed a large, dense mass in the right infratemporal fossa (Fig. i a, b). The lesion extended into the pterygopalatine space, bowed the lateral wall of the maxillary sinus anteriorly and eroded the floor of the middle cranial fossa and lateral pterygoid plate (Fig. 1 c). Magnetic resonance imaging (MRI) showed multiple areas of signal void, sug-
ASPS is a rare soft tissue sarcoma of unknown origin, affecting mainly children and young adults (age range: 1130 years) [2]. Although female predominance has been noted in some series, others report equal sex distribution [3, 4]. The right side of the body is, however, affected more commonly [5]. More than 60 % of ASPS occur in the buttocks or thighs [6]. The head and neck are not uncommon sites; most ASPS in this area are found in the orbit or tongue [7]. Other sites include the retroperitoneum, perineum, female genital tract, and the abdomen. At the time of diagnosis most tumors exceed 5 cm in size, but most are asymptomatic; pulmonary metastases are not an uncommon initial presentation [8]. Histologically, ASPS has been considered to be a variant of paraganglioma, neural or skeletal tumor, or a modified skeletal muscle neoplasm. A pseudoalveolor (organoid) pattern characterized by aggregates of cells surrounded by thin-walled blood vessels and fibrous septa is present. Intracytoplasmic periodic acid-Schiff-positive crystals are distinctive and are found in 20-85 % of cases. Mitoses and pleomorphism are rare [2]. Despite this, ASPS is one of the most malignant sarcomas. The tumor recurs locally in 20-30 % of cases; metastases will develop in 40-70 % of patients [1], the lungs, bone, and brain being the most common sites. Five-year survival varies between 40 and 60 % [1].
368
Fig.la, b. Contrast-enhanced CT through the skull base. a Soft tissue, b bone algorithm axial sections. A large, enhancing mass obliterates the muscle planes in the right infratemporal fossa and extends to the pterygopalatine fossa (arrow). Bowing of the lateral wall of the right maxillary sinus indicates slow growth of the tumor. The right pterygoid bone is markedly osteopenic. c Coronal section (bone algorithm). The tumor erodes the floor of the middle cranial fossa (large arrows) and the lateral pterygoid plate (small arrows) Fig.2. a Axial T2-weighted MR image (SE 2000/80) demonstrates a hyperintense mass in the right infratemporal fossa. Multiple signal voids within the mass suggest the presence of vessels. b, c Tl-weighted MRI (SE 500/25) immediately after intravenous gadolinium. b Axial section at the same level as ~u c Coronal section. The mass enhances richly and extends to the dura mater (arrows) of the right middle cranial fossa
Fig. 3. Lateral subtraction angiogram (anterior on left) shows a markedly hypervascular tumor fed by a hypertrophied internal maxillary artery
To o u r k n o w l e d g e , C T findings in A S P S have b e e n r e p o r t e d in onyl two cases [9, 10]. I n the first, C T showed a h y p e r v a s c u l a r mass with n u m e r o u s d i l a t e d vessels in the thigh of a y o u n g f e m a l e [9], while the s e c o n d was a n o n - s p e c i f i c r e t r o b u l b a r mass in a y o u n g girl [10]. This is the first d e s c r i p t i o n of M R I findings in this rare tumor.
References 1. Lieberman PH, Brennan MF, Kimmel M, Erlandson RA, Garin-Chesa P, Flehinger BY (1989) Alveolar soft-part sarcoma. A clinico-pathologicstudy of half a century. Cancer 63: 113 2. Evans HL (1985) Alveolar soft-part sarcoma: a study of 13 typical examples and one with histologically atypical component. Cancer 55:912-917 3. Chapman GW, Benda J, Williams T (1984) Alveolar-soft-part sarcoma of the vagina. Gynecol Onco118:125-129 4. Shen JT, D'Ablaing G, Morrow CP (1982) Alveolar soft part sarcoma of the vulva: report of first case and review of literature. Gynecol Oncol 13:120-128
369 5. Rubinstein MI, Drake AE McClatchey KD (1988) Alveolar soft part sarcoma of the nasal cavity: report of a case and a review of the literature. Laryngoscope 98:1246-1250 6. Auerbach HE, Brooks JJ (1987) Alveolar soft part sarcoma: a clinicopathologic and immunohistochemical study. Cancer 60: 66-73 7. Spector RA, Travis LW, Smith J (1987) Alveolar soft part sarcoma of the head and neck. Laryngosocpe 89:1301-1306 8. Font RL, Jurco S, Zimmerman LE (1982) Alveolar soft part sarcoma of the orbit: a clinicopathologicanalysis of seventeen cases and a review of the literature. Hum Patho113: 569-579
9. Radin DR, Ralls PW, Boswell WD, et al (1984) Alveolar soft part sarcoma: CT findings. J Comput Assist Tomogr 8:344-345 10. Grant GD, Shields JA, Flanagan JC, Horowitz P (1979) The ultrasonographic and radiologic features of a histologically proven case of alveolar soft part sarcoma of the orbit. Am J Ophthalmo187:773-777 M. Castillo, M. D. Section of Neuroradiology, CB 7510 UNC-CH Chapel Hill, NC 27599-7510, USA
Neuroradiology (1992) 34:36%369
Head and Neck Radiology
9 Springer-Verlag 1992
Infratemporal alveolar soft part sarcoma: CT, MRI and angiographic findings M. Castillo t , Y. Y. Lee 2, and S. Yamasaki 2
1Sections of Neuroradiology, University of Texas Medical School, L. B.J. General Hospital, and 2University of Texas System Cancer Center, M. D. Anderson Hospital, Houston, Texas, USA Received: 15 April 1991
Summary. The imaging features of a rare alveolar soft part sarcoma found in a 44-year-old female are presented. Although the tumor showed hypervascularity by angiography, CT and MRI suggested slow growth. Despite this relatively benign appearance, alveolar soft part sarcoma is one of the most malignant sarcomas.
gesting vessels, and intense enhancement following the administration of gadolinium-DTPA (Fig.2 a,b). The tumor invaded the dura mater of the right middle cranial fossa (Fig.2 c). At angiography, the hypervascular mass was mainly supplied by an enlarged internal maxillary artery (Fig. 3). It was embolized with Gelfoam and then resected entirely. Pathology examination showed the tumor to be an ASPS.
Key words: Alveolar soft part sarcoma - Soft tissue sarcoma - Sarcoma Discussion
Alveolar soft part sarcoma (ASPS) is an extremely rare tumor accounting for less than 1% of all soft tissue sarcomas. More than half of ASPS occur in the muscles or fascial planes of the lower limbs [1]. We present a case of ASPS presenting in the infratemporal fossa.
Case report
A 44-year-old female presented with a 3-month history of progressive right-sided trismus and pulsatile tinnitus. A year previously she had noted right scalp tenderness which slowly progressed to dysesthesiae of the right side of the face and tongue. Examination revealed diffuse swelling of the right side of the face extending to the preauricular and temporal regions, mild trismus and a mucosa-covered mass in the roof of the right nasopharynx. Contrast-enhanced computed tomography (CT) showed a large, dense mass in the right infratemporal fossa (Fig. i a, b). The lesion extended into the pterygopalatine space, bowed the lateral wall of the maxillary sinus anteriorly and eroded the floor of the middle cranial fossa and lateral pterygoid plate (Fig. 1 c). Magnetic resonance imaging (MRI) showed multiple areas of signal void, sug-
ASPS is a rare soft tissue sarcoma of unknown origin, affecting mainly children and young adults (age range: 1130 years) [2]. Although female predominance has been noted in some series, others report equal sex distribution [3, 4]. The right side of the body is, however, affected more commonly [5]. More than 60 % of ASPS occur in the buttocks or thighs [6]. The head and neck are not uncommon sites; most ASPS in this area are found in the orbit or tongue [7]. Other sites include the retroperitoneum, perineum, female genital tract, and the abdomen. At the time of diagnosis most tumors exceed 5 cm in size, but most are asymptomatic; pulmonary metastases are not an uncommon initial presentation [8]. Histologically, ASPS has been considered to be a variant of paraganglioma, neural or skeletal tumor, or a modified skeletal muscle neoplasm. A pseudoalveolor (organoid) pattern characterized by aggregates of cells surrounded by thin-walled blood vessels and fibrous septa is present. Intracytoplasmic periodic acid-Schiff-positive crystals are distinctive and are found in 20-85 % of cases. Mitoses and pleomorphism are rare [2]. Despite this, ASPS is one of the most malignant sarcomas. The tumor recurs locally in 20-30 % of cases; metastases will develop in 40-70 % of patients [1], the lungs, bone, and brain being the most common sites. Five-year survival varies between 40 and 60 % [1].
368
Fig.la, b. Contrast-enhanced CT through the skull base. a Soft tissue, b bone algorithm axial sections. A large, enhancing mass obliterates the muscle planes in the right infratemporal fossa and extends to the pterygopalatine fossa (arrow). Bowing of the lateral wall of the right maxillary sinus indicates slow growth of the tumor. The right pterygoid bone is markedly osteopenic. c Coronal section (bone algorithm). The tumor erodes the floor of the middle cranial fossa (large arrows) and the lateral pterygoid plate (small arrows) Fig.2. a Axial T2-weighted MR image (SE 2000/80) demonstrates a hyperintense mass in the right infratemporal fossa. Multiple signal voids within the mass suggest the presence of vessels. b, c Tl-weighted MRI (SE 500/25) immediately after intravenous gadolinium. b Axial section at the same level as ~u c Coronal section. The mass enhances richly and extends to the dura mater (arrows) of the right middle cranial fossa
Fig. 3. Lateral subtraction angiogram (anterior on left) shows a markedly hypervascular tumor fed by a hypertrophied internal maxillary artery
To o u r k n o w l e d g e , C T findings in A S P S have b e e n r e p o r t e d in onyl two cases [9, 10]. I n the first, C T showed a h y p e r v a s c u l a r mass with n u m e r o u s d i l a t e d vessels in the thigh of a y o u n g f e m a l e [9], while the s e c o n d was a n o n - s p e c i f i c r e t r o b u l b a r mass in a y o u n g girl [10]. This is the first d e s c r i p t i o n of M R I findings in this rare tumor.
References 1. Lieberman PH, Brennan MF, Kimmel M, Erlandson RA, Garin-Chesa P, Flehinger BY (1989) Alveolar soft-part sarcoma. A clinico-pathologicstudy of half a century. Cancer 63: 113 2. Evans HL (1985) Alveolar soft-part sarcoma: a study of 13 typical examples and one with histologically atypical component. Cancer 55:912-917 3. Chapman GW, Benda J, Williams T (1984) Alveolar-soft-part sarcoma of the vagina. Gynecol Onco118:125-129 4. Shen JT, D'Ablaing G, Morrow CP (1982) Alveolar soft part sarcoma of the vulva: report of first case and review of literature. Gynecol Oncol 13:120-128
369 5. Rubinstein MI, Drake AE McClatchey KD (1988) Alveolar soft part sarcoma of the nasal cavity: report of a case and a review of the literature. Laryngoscope 98:1246-1250 6. Auerbach HE, Brooks JJ (1987) Alveolar soft part sarcoma: a clinicopathologic and immunohistochemical study. Cancer 60: 66-73 7. Spector RA, Travis LW, Smith J (1987) Alveolar soft part sarcoma of the head and neck. Laryngosocpe 89:1301-1306 8. Font RL, Jurco S, Zimmerman LE (1982) Alveolar soft part sarcoma of the orbit: a clinicopathologicanalysis of seventeen cases and a review of the literature. Hum Patho113: 569-579
9. Radin DR, Ralls PW, Boswell WD, et al (1984) Alveolar soft part sarcoma: CT findings. J Comput Assist Tomogr 8:344-345 10. Grant GD, Shields JA, Flanagan JC, Horowitz P (1979) The ultrasonographic and radiologic features of a histologically proven case of alveolar soft part sarcoma of the orbit. Am J Ophthalmo187:773-777 M. Castillo, M. D. Section of Neuroradiology, CB 7510 UNC-CH Chapel Hill, NC 27599-7510, USA
