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Mazabraud's syndrome coexisting with a uterine tumor resembling an ovarian sex cord tumor (UTROSCT): a case report.

Calisir C, Inan U, Yavas US, Isiksoy S, Kaya T - Korean J Radiol (2007 Sep-Oct)

Bottom Line: The association of intramuscular myxoma and fibrous dysplasia is a rare disease known as Mazabraud's syndrome.We present a case of Mazabraud's syndrome coexisting with a uterine tumor and resembling an ovarian sex cord tumor (UTROSCT).To the best of our knowledge, the coexistence of the two different entities has not been reported in the literature.

View Article: PubMed Central - PubMed

Affiliation: Department of Radiology, Eskisehir Osmangazi University. cuneytcalisir_72@yahoo.com

ABSTRACT
The association of intramuscular myxoma and fibrous dysplasia is a rare disease known as Mazabraud's syndrome. We present a case of Mazabraud's syndrome coexisting with a uterine tumor and resembling an ovarian sex cord tumor (UTROSCT). This uterine tumor showed a high mitotic index and cytological atypia. To the best of our knowledge, the coexistence of the two different entities has not been reported in the literature.

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Related in: MedlinePlus

A, B. Coronal T1-weighted (A) and coronal STIR MR images (B) demonstrate an area of signal abnormality within the left ilium. The region appears with low signal intensity on T1 and high signal intensity on STIR relative to adult yellow marrow, consistent with fibrous dysplasia (arrow). Also identified are multiple intramuscular masses within the left gluteal musculature and left thigh, which appear with low signal intensity on T1-weighted images and high signal intensity on STIR images relative to muscle, consistent with myxomas (arrowheads).C, D. An axial T1-weighted image (C) Axial T2-weighted MR images (D) demonstrate an oval, sharply defined mass in the left gluteus maximus, which has a low signal intensity on T1 and high signal intensity on T2 with homogeneity in both signals (arrows).E. An anteroposterior radiograph of the left hip demonstrates a well-defined oval osteolytic lesion with a thin, sclerotic rim within the left ilium (arrows).F. Ultrasonography of the left gluteal musculature shows a heterogeneous, solid, hypoechoic, lobulated intramuscular tumor with multiple small-sized fluid filled cystic areas.
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Figure 1: A, B. Coronal T1-weighted (A) and coronal STIR MR images (B) demonstrate an area of signal abnormality within the left ilium. The region appears with low signal intensity on T1 and high signal intensity on STIR relative to adult yellow marrow, consistent with fibrous dysplasia (arrow). Also identified are multiple intramuscular masses within the left gluteal musculature and left thigh, which appear with low signal intensity on T1-weighted images and high signal intensity on STIR images relative to muscle, consistent with myxomas (arrowheads).C, D. An axial T1-weighted image (C) Axial T2-weighted MR images (D) demonstrate an oval, sharply defined mass in the left gluteus maximus, which has a low signal intensity on T1 and high signal intensity on T2 with homogeneity in both signals (arrows).E. An anteroposterior radiograph of the left hip demonstrates a well-defined oval osteolytic lesion with a thin, sclerotic rim within the left ilium (arrows).F. Ultrasonography of the left gluteal musculature shows a heterogeneous, solid, hypoechoic, lobulated intramuscular tumor with multiple small-sized fluid filled cystic areas.

Mentions: A physical examination demonstrated a large, painless and mobile mass at the area of concern. Magnetic resonance imaging (MRI) scans of the left hip and gluteal area including axial and coronal T1 weighted spin-echo (SE) images (TR: 582 ms, TE: 16 ms) and turbo short tau inversion recovery (STIR, TR 3000 ms, TE 18 ms, TI 100) images were obtained on a 1-T unit with a body coil. In addition axial T2 weighted turbo-spin-echo (TSE) imaging (TR: 3000 ms, TE: 112 ms) was included. These studies demonstrated at least four lobulated soft tissue masses located within the left gluteal musculature, a soft tissue mass within the anterolateral musculature of the left thigh and a solitary bone lesion involving the left ilium. The soft tissue masses were markedly hypointense compared with that of skeletal muscle on T1-weighted images and hyperintense on T2 weighted and STIR images. The bone lesion showed decreased signal intensity on T1-weighted images and increased signal intensity on T2 weighted and STIR images (Figs. 1A-D). Radiography of the pelvis demonstrated a solitary, osteolytic lesion with thin sclerotic margins within the left ilium, which is consistent with the presence of fibrous dysplasia (Fig. 1E). Ultrasonography of the symptomatic and the largest left gluteal mass showed a 4.5 × 4 × 4 cm lobulated, solid, heterogeneous, hypoechoic tumor with multiple small-sized fluid filled cystic areas (Fig. 1F). A differential diagnosis at the time included soft tissue sarcomas and soft tissue metastases. Magnetic resonance imaging also revealed an enlarged uterus and endometrial cavity completely occupied by a large mass that measured approximately 11 cm in the transverse diameter. The mass showed intermediate signal intensity on STIR and low signal intensity on T1 weighted images (Figs. 2A, B). In laboratory studies, the CA 125 level was higher than normal. FDG-PET showed accumulation of FDG in the lesion located in the uterus. No accumulation was observed in the solitary bone lesion located within the left ilium and soft tissue masses (Fig. 2C). The patient underwent excisional biopsy of the symptomatic and the largest left gluteal mass. At the time of surgery, the tumor was found to be located within the gluteus maximus muscle. The lesion was completely excised, including a small cuff of normal appearing musculature. Grossly, the tumor measured 5 × 4 × 4 cm in dimension and appeared as a lobulated ovoid cystic mass that was partially covered by and adherent to skeletal muscle. The cut surface of the tumor revealed many cystic spaces (size ranged from 1 to 2.5 cm), which were filled with mucoid material, and solid gelatinous areas. A histological evaluation showed an intramuscular myxoma, with no sign of malignancy (Fig. 3). An abnormal left ilium and soft tissue myxomas indicated a typical case of Mazabraud's syndrome. Later, a total abdominal hysterectomy and bilateral salpingo-oophorectomy were performed because of the tumor of the uterus. Macroscopically, the tumor grew as a nodular mass within the myometrium and was 8.5 cm in diameter. On a histological examination, the tumor was composed entirely of epithelioid cells displaying sex cord-like structures as cords, anastomosing trabecula, and solid sheets of cells. Tumor cells had plump vesicular nuclei with prominent nucleoli. The stroma was scanty, and was seen as fibrous or hyalinized bands between cell groups. Nine mitoses/10 high power fields were determined (Fig. 4).


Mazabraud's syndrome coexisting with a uterine tumor resembling an ovarian sex cord tumor (UTROSCT): a case report.

Calisir C, Inan U, Yavas US, Isiksoy S, Kaya T - Korean J Radiol (2007 Sep-Oct)

A, B. Coronal T1-weighted (A) and coronal STIR MR images (B) demonstrate an area of signal abnormality within the left ilium. The region appears with low signal intensity on T1 and high signal intensity on STIR relative to adult yellow marrow, consistent with fibrous dysplasia (arrow). Also identified are multiple intramuscular masses within the left gluteal musculature and left thigh, which appear with low signal intensity on T1-weighted images and high signal intensity on STIR images relative to muscle, consistent with myxomas (arrowheads).C, D. An axial T1-weighted image (C) Axial T2-weighted MR images (D) demonstrate an oval, sharply defined mass in the left gluteus maximus, which has a low signal intensity on T1 and high signal intensity on T2 with homogeneity in both signals (arrows).E. An anteroposterior radiograph of the left hip demonstrates a well-defined oval osteolytic lesion with a thin, sclerotic rim within the left ilium (arrows).F. Ultrasonography of the left gluteal musculature shows a heterogeneous, solid, hypoechoic, lobulated intramuscular tumor with multiple small-sized fluid filled cystic areas.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2626811&req=5

Figure 1: A, B. Coronal T1-weighted (A) and coronal STIR MR images (B) demonstrate an area of signal abnormality within the left ilium. The region appears with low signal intensity on T1 and high signal intensity on STIR relative to adult yellow marrow, consistent with fibrous dysplasia (arrow). Also identified are multiple intramuscular masses within the left gluteal musculature and left thigh, which appear with low signal intensity on T1-weighted images and high signal intensity on STIR images relative to muscle, consistent with myxomas (arrowheads).C, D. An axial T1-weighted image (C) Axial T2-weighted MR images (D) demonstrate an oval, sharply defined mass in the left gluteus maximus, which has a low signal intensity on T1 and high signal intensity on T2 with homogeneity in both signals (arrows).E. An anteroposterior radiograph of the left hip demonstrates a well-defined oval osteolytic lesion with a thin, sclerotic rim within the left ilium (arrows).F. Ultrasonography of the left gluteal musculature shows a heterogeneous, solid, hypoechoic, lobulated intramuscular tumor with multiple small-sized fluid filled cystic areas.
Mentions: A physical examination demonstrated a large, painless and mobile mass at the area of concern. Magnetic resonance imaging (MRI) scans of the left hip and gluteal area including axial and coronal T1 weighted spin-echo (SE) images (TR: 582 ms, TE: 16 ms) and turbo short tau inversion recovery (STIR, TR 3000 ms, TE 18 ms, TI 100) images were obtained on a 1-T unit with a body coil. In addition axial T2 weighted turbo-spin-echo (TSE) imaging (TR: 3000 ms, TE: 112 ms) was included. These studies demonstrated at least four lobulated soft tissue masses located within the left gluteal musculature, a soft tissue mass within the anterolateral musculature of the left thigh and a solitary bone lesion involving the left ilium. The soft tissue masses were markedly hypointense compared with that of skeletal muscle on T1-weighted images and hyperintense on T2 weighted and STIR images. The bone lesion showed decreased signal intensity on T1-weighted images and increased signal intensity on T2 weighted and STIR images (Figs. 1A-D). Radiography of the pelvis demonstrated a solitary, osteolytic lesion with thin sclerotic margins within the left ilium, which is consistent with the presence of fibrous dysplasia (Fig. 1E). Ultrasonography of the symptomatic and the largest left gluteal mass showed a 4.5 × 4 × 4 cm lobulated, solid, heterogeneous, hypoechoic tumor with multiple small-sized fluid filled cystic areas (Fig. 1F). A differential diagnosis at the time included soft tissue sarcomas and soft tissue metastases. Magnetic resonance imaging also revealed an enlarged uterus and endometrial cavity completely occupied by a large mass that measured approximately 11 cm in the transverse diameter. The mass showed intermediate signal intensity on STIR and low signal intensity on T1 weighted images (Figs. 2A, B). In laboratory studies, the CA 125 level was higher than normal. FDG-PET showed accumulation of FDG in the lesion located in the uterus. No accumulation was observed in the solitary bone lesion located within the left ilium and soft tissue masses (Fig. 2C). The patient underwent excisional biopsy of the symptomatic and the largest left gluteal mass. At the time of surgery, the tumor was found to be located within the gluteus maximus muscle. The lesion was completely excised, including a small cuff of normal appearing musculature. Grossly, the tumor measured 5 × 4 × 4 cm in dimension and appeared as a lobulated ovoid cystic mass that was partially covered by and adherent to skeletal muscle. The cut surface of the tumor revealed many cystic spaces (size ranged from 1 to 2.5 cm), which were filled with mucoid material, and solid gelatinous areas. A histological evaluation showed an intramuscular myxoma, with no sign of malignancy (Fig. 3). An abnormal left ilium and soft tissue myxomas indicated a typical case of Mazabraud's syndrome. Later, a total abdominal hysterectomy and bilateral salpingo-oophorectomy were performed because of the tumor of the uterus. Macroscopically, the tumor grew as a nodular mass within the myometrium and was 8.5 cm in diameter. On a histological examination, the tumor was composed entirely of epithelioid cells displaying sex cord-like structures as cords, anastomosing trabecula, and solid sheets of cells. Tumor cells had plump vesicular nuclei with prominent nucleoli. The stroma was scanty, and was seen as fibrous or hyalinized bands between cell groups. Nine mitoses/10 high power fields were determined (Fig. 4).

Bottom Line: The association of intramuscular myxoma and fibrous dysplasia is a rare disease known as Mazabraud's syndrome.We present a case of Mazabraud's syndrome coexisting with a uterine tumor and resembling an ovarian sex cord tumor (UTROSCT).To the best of our knowledge, the coexistence of the two different entities has not been reported in the literature.

View Article: PubMed Central - PubMed

Affiliation: Department of Radiology, Eskisehir Osmangazi University. cuneytcalisir_72@yahoo.com

ABSTRACT
The association of intramuscular myxoma and fibrous dysplasia is a rare disease known as Mazabraud's syndrome. We present a case of Mazabraud's syndrome coexisting with a uterine tumor and resembling an ovarian sex cord tumor (UTROSCT). This uterine tumor showed a high mitotic index and cytological atypia. To the best of our knowledge, the coexistence of the two different entities has not been reported in the literature.

Show MeSH
Related in: MedlinePlus