n Case 201
Note: This copy is for your personal non-commercial use only. To order presentation-ready copies for distribution to your colleagues or clients, contact us at www.rsna.org/rsnarights.
Diagnosis Please
Case 201: Glomus Tumor of the Breast1
Mona El Khoury, MD Danh Tran-Tranh, MD Donato Terrone, MD Julie David, MD Lucie Lalonde, MD Isabelle Trop, MD
History
A 55-year-old woman presented to our breast clinic with 1-year history of lancinating pain in the right breast that was exacerbated by blunt pressure and cold temperature. She denied any family history of breast cancer or any personal history of trauma, infection, or surgery of the breast. On clinical examination, exquisite pain was generated by simple light pressure over the upper outer quadrant of the right breast. Mammography and ultrasounography (US) with Doppler analysis of the right breast were painful but were performed with the least pressure possible.
Imaging Findings
Part one of this case appeared 4 months previously and may contain larger images. Published online 10.1148/radiol.13120919 Content code: Radiology 2014; 270:302–306 1 From
the Departments of Radiology (M.E.K., D. Terrone, J.D., L.L., I.T.) and Pathology (D. Tran-Tranh), Centre Hospitalier Universitaire de l’Université de Montréal, 3840 Rue Saint Urbain, Montréal, QC, Canada H2W 1T8. Received May 8, 2012; revision requested June 8; revision received August 2; accepted August 3; final version accepted August 22. Address correspondence to M.E.K. (e-mail: elkhourymona @yahoo.fr). Conflicts of interest are listed at the end of this article. q RSNA, 2014
302
Discussion
Bilateral mammograms (Fig 1) revealed an oval hyperdense mass in the upper outer quadrant of the right breast, with partially obscured margins and no microcalcifications. US examination of the right breast (Fig 2a) was extremely painful. Exquisite pain was generated when the probe precisely overlaid an oval, hypoechoic, well-circumscribed mildly lobulated superficially located mass that correlated with the mammographic finding. At color Doppler analysis and spectral assessment, this mass had arterial and venous vessels without shunting and an efferent vessel (Fig 2b, 2c). The patient underwent surgical excision. Pathologic analysis (Fig 3) revealed the presence of a well-defined mass within the fibroadipose tissue characterized by a proliferation of vascular structures surrounded by small monomorphic uniform cells with round nuclei and eosinophilic cytoplasm. The center of the lesion displayed myxoid changes. No substantial mitotic activity or cytologic atypia were noted. At immunohistochemical analy sis, the round uniform cells stained positive for smooth muscle actin and vimentin, weakly positive for CD34, and negative for keratin AE1 and AE3, CD31, and desmin.
The patient’s clinical presentation with chronic pain in the right breast and a pinpoint exquisite tenderness over a superficially located hypervascular mass was unusual but virtually pathognomonic for glomus tumor. The diagnosis was confirmed after surgical excision and pathologic analysis. Glomus tumors are uncommon, extremely painful usually benign mesenchymal neoplasms. They arise from the glomus body, which is a specialized neurovascular organ composed of an afferent arteriole, anastomotic vessel (Sucquet-Hoyer canal), efferent collecting vein, intraglomerular reticulum, and capsular portion. The Sucquet-Hoyer canal is surrounded by endothelial cells and spherical uniform glomus cells thought to be modified smooth muscle cells (1–3). The normal glomus body acts as a contractile neuromyoarterial receptor that regulates peripheral blood flow and is thus involved in temperature and blood pressure regulation. Normal glomus bodies are present in the deepest layer of the dermis (ie, the reticular dermis) throughout the body (2). Histologically, glomus tumors are formed, as a rule, by uniform round
radiology.rsna.org n Radiology: Volume 270: Number 1—January 2014
DIAGNOSIS PLEASE: Glomus Tumor of the Breast
El Khoury et al
Figure 1
Figure 1: (a) Craniocaudal and (b) mediolateral oblique mammograms of the right breast show an oval-shaped hyperdense mass with partially obscured margins in the upper outer quadrant (arrow). Note the mammographically visible nipple markers.
cells with distinct cell borders and a prominent capillary vasculature at the periphery of the mass. They are classified as glomangiomas when their vascular structures are prominent (4), and they may be mistaken for cutaneous round cell tumors or sweat gland tumors when the glomus cells in the tumor are numerous and overshadow the vascular component (5). The high velocity flow within the intratumoral vessels accounts for the high vascularity noted at color Doppler analysis, which is specific for the diagnosis (6). Glomus tumors should be distinguished from chemodectomas (paragangliomas), which may also be designated as glomus tumors or glomus jugular tumors but are different entities that originate from paraganglion cells of the parasympathetic system (5).
Glomus tumors are typically solitary, small, and encountered superficially in the extremities, mainly the subungual areas. They may, however, be atypical, multiple, large, or infiltrative, and they can occur anywhere in the body, including in the gastrointestinal and genitourinary tracts, lung, mediastinum, liver, pancreas, ovary, bone, and soft tissue (1). To our knowledge, only two cases of glomus tumor have been previously reported in the breast (one in a male patient [7] and one in a female patient [8]) and two cases have been reported in the axilla (9,10). When cutaneous, glomus tumors are usually benign, encapsulated, smaller than 1 cm in diameter, and manifest as a blue-red nodule with characteristic paroxysmal pain and sensitivity to cold. This triad of pain, tenderness, and sen-
Radiology: Volume 270: Number 1—January 2014 n radiology.rsna.org
sitivity to cold is known to be pathognomonic of glomus tumors (8) and enables distinction of this rare entity from the other more frequently encountered superficial breast masses, including hemangiomas, angiolipomas, angiosarcomas, arteriovenous malformations, false aneurysms, hemangiopericytomas, and granular cell tumors. Hemangioma is the most frequent benign vascular tumor in the breast. It typically manifests as a hypoechoic well-defined oval mass, and it may be of the cavernous or capillary type depending on the size of its vascular channels (11). It may be hypervascular at color Doppler US, but unlike glomus tumors, it has a more superficial location in the papillary dermis or the epidermis and is not painful (6). Angiolipoma is a fat-containing benign tumor admixed with vascular prolif303
DIAGNOSIS PLEASE: Glomus Tumor of the Breast
El Khoury et al
Figure 2
Figure 2: (a) Radial US image of the right breast shows an oval-shaped well-marginated mildly lobulated and superficially located mass (arrows). (b) Radial US color Doppler image shows internal vascularity with turbulent arterial and venous blood flow and an efferent vessel. (c) Radial US spectral Doppler image shows high velocity arterial flow within the mass.
Figure 3
Figure 3: (a, b) Histopathologic slices stained with hematoxylin-eosin (original magnification, 320 and 3200 in a and b, respectively) show nodular proliferation of small round cells with vascular structures (arrows), which is in keeping with a glomus tumor.
erations and is typically hyperechoic (12). It may be painful to palpation. The pain often diminishes over time, and un304
like in glomus tumors, it is not intensified by thermal changes. The vessels in an angiolipoma are small-caliber capil-
laries, often containing thrombi and surrounded by fat. They do not show any tendency for malignant transformation
radiology.rsna.org n Radiology: Volume 270: Number 1—January 2014
DIAGNOSIS PLEASE: Glomus Tumor of the Breast
or local recurrence when surgically excised (13). Angiosarcoma may be primary or secondary, associated with previous radiation therapy or lymphedema, and may show, as glomus tumors, a blue skin discoloration and high vascularity on Doppler US images; however, it manifests as an ill-defined mass with skin thickening (12,14). Thus, it is not the most likely diagnosis in this patient given the clinical and imaging findings of a well-defined mass. Hemangiopericytoma is a benign vascular tumor with a potentially malignant nature originating from the capillary pericytes, which are the contractile cells surrounding the walls of the vessels. Hemangiopericytoma can be hypervascular on color Doppler images, with a feeding vessel (15,16). False aneurysm or communicating hematoma surrounded by the adventitia of an arterial vessel and perivascular tis sue may manifest as an anechoic ovoid mass with turbulent flow on color Doppler images; however, it displays the typical yin-yang appearance and occurs in the setting of acute trauma and is often pulsatile (12). Granular cell tumors arise from Schwann cells of peripheral nerves. About 5%–8% of these tumors occur in the breast, usually from the dermis, and project into subcutaneous tissue. They may manifest as a mass with either well-circumscribed or spiculated and infiltrative margins usually associated with overlying skin thickening or retraction (14,17). Amid all these superficially located breast masses with different degree of vascularity, only glomus tumors are associated with exquisite pain. Very rare cases of malignant glomus tumors have been reported. Folpe et al (18) reviewed 52 unusual cases of glomus tumors and classified them as (a) malignant, with high risk of metastasis when deeply located and larger than 2 cm or when displaying atypical mitotic figures or moderate to high nuclear grade and more than five mitotic figures per 50 high-power fields; (b) symplastic when displaying a high nuclear grade and no other malignant feature; (c) of
El Khoury et al
uncertain malignant potential when lacking firm criteria for malignancy or benignancy, and (d) glomangiomatosis when they have the histologic features of diffuse angiomatosis and excess glomus cells. Immunohistochemical analysis helps establish the correct diagnosis, since glomus tumors stain negative for desmin, cytokeratin, and S100 and positive for vimentin and smooth muscle actin (18). Unlike glomus tumors, melanomas, Merckel cell carcinomas, and eccrine spireadenomas express S100 and cytokeratin, whereas the extremely rare primitive neuroectodermal tumors and neuroblastomas do not express muscle actin (18). When a glomus tumor is suspected in the breast, US is the first imaging technique preformed because patients usually are unable to tolerate compression because of severe pain (6). At imaging, glomus tumors typically manifest as an extremely painful, hypervascular well-marginated superficial mass (6). The treatment of choice is surgical excision (6). If inadequately excised, glomus tumors—particularly subungual ones, may recur in 1%–2% of cases (2,7). In summary, glomus tumors that develop outside the more typical subungual location, including those that develop in the breasts, are uncommon and present a diagnostic challenge. The most important initial step for making the correct diagnosis is clinical suspicion in a patient who presents with chronic local pain, dysesthesia, and pinpoint tenderness to pressure anywhere in the body, including the breast, as in this patient. By incorporating the clinical presentation with imaging findings (mainly US findings), the breast radiologist can effectively establish the diagnosis with no need for percutaneous biopsy, which is generally intolerable due to extreme pain. Disclosures of Conflicts of Interest: M.E.K. No relevant conflicts of interest to disclose. D. Tran-Thanh No relevant conflicts of interest to disclose. D. Terrone No relevant conflicts of interest to disclose. J.D. No relevant conflicts of interest to disclose. L.L. No relevant conflicts
Radiology: Volume 270: Number 1—January 2014 n radiology.rsna.org
of interest to disclose. I.T. No relevant conflicts of interest to disclose.
References 1. Vigovich FA, Hurt MA, Santa Cruz DJ. Sclerotic glomus tumor. Am J Dermatopathol 2010;32(1):76–78. 2. Drapé JL, Idy-Peretti I, Goettmann S, et al. Subungual glomus tumors: evaluation with MR imaging. Radiology 1995;195(2):507–515. 3. Hakverdi S, Kalaci A, Yaldiz M, Akansu B. Glomus tumor of the hip: an unusual location. Saudi Med J 2009;30(2):299–301. 4. Jaiswal VR, Champine JG, Sharma S, Molberg KH. Primary glomangioma of the liver: a case report and review of the literature. Arch Pathol Lab Med 2004;128(3):e46–e49. 5. Ghaly RF, Ring AM. Supraclavicular glomus tumor, 20 year history of undiagnosed shoulder pain: a case report. Pain 1999;83 (2):379–382. 6. Baek HJ, Lee SJ, Cho KH, et al. Subungual tumors: clinicopathologic correlation with US and MR imaging findings. RadioGraphics 2010;30(6):1621–1636. 7. Iuanow E, Kettler M, Slanetz PJ. Spectrum of disease in the male breast. AJR Am J Roentgenol 2011;196(3):W247–W259. 8. Yalcin S, Ergul E, Ucar AE, Ulger BV, Korukluoglu B. Glomus tumor of the breast: first report. Langenbecks Arch Surg 2009; 394(2):399–400. 9. Gould EW, Manivel JC, Albores-Saavedra J, Monforte H. Locally infiltrative glomus tumors and glomangiosarcomas: a clinical, ultrastructural, and immunohistochemical study. Cancer 1990;65(2):310–318. 10. Holck S, Bredesen JL. Solid glomus tumor presenting as an axillary mass: report of a case with morphologic study, including cytologic characteristics. Acta Cytol 1996;40(3): 555–562. 11. Mesurolle B, Sygal V, Lalonde L, et al. Sonographic and mammographic appearances of breast hemangioma. AJR Am J Roentgenol 2008;191(1):W17–W22. 12. Jesinger RA, Lattin GE Jr, Ballard EA, Zelasko SM, Glassman LM. Vascular abnormalities of the breast: arterial and venous disorders, vascular masses, and mimic lesions with radiologic-pathologic correlation. RadioGraphics 2011;31(7):E117–E136. 13. Murphey MD, Carroll JF, Flemming DJ, Pope TL, Gannon FH, Kransdorf MJ. From the archives of the AFIP: benign musculoskeletal lipomatous lesions. RadioGraphics 2004;24(5):1433–1466.
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DIAGNOSIS PLEASE: Glomus Tumor of the Breast
14. Kalli S, Freer PE, Rafferty EA. Lesions of the skin and superficial tissue at breast MR imaging. RadioGraphics 2010;30(7):1891–1913. 15. van Kints MJ, Tham RT, Klinkhamer PJ, van den Bosch HC. Hemangiopericytoma of the breast: mammographic and sonographic findings. AJR Am J Roentgenol 1994;163(1):61–63. 16. Vilanova JC, Barceló J, Smirniotopoulos JG, et al. Hemangioma from head to toe: MR imaging with pathologic correlation. RadioGraphics 2004;24(2):367–385. 17. Giess CS, Raza S, Birdwell RL. Distinguishing breast skin lesions from superficial breast parenchymal lesions: diagnostic criteria, imaging characteristics, and pitfalls. RadioGraphics 2011;31(7):1959–1972. 18. Folpe AL, Fanburg-Smith JC, Miettinen M, Weiss SW. Atypical and malignant glomus tumors: analysis of 52 cases, with a proposal for the reclassification of glomus tumors. Am J Surg Pathol 2001;25(1):1–12.
Congratulations to the 63 individuals and two resident groups that submitted the most likely diagnosis (glomus tumor of the breast) for Diagnosis Please, Case 201. The names and locations of the individuals and resident groups, as submitted, are as follows:
Individual responses Albert J. Alter, MD, PhD, Blanchardville, Wis Sebastian Bravo-Grau, MD, MSc, Santiago, Region Metropolitana, Chile Ian A. Burgess, MD, North Sydney, New South Wales, Australia Michael H. Childress, MD, Silver Spring, Md
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El Khoury et al
Christopher Chu, MBBS, FRANZC, Sydney, New South Wales, Australia Theresa M. Corrigan, MD, Louisville, Ky Jezreel C. Da Costa, Porto Velho, Brazil Anil K. Dasyam, MD, Pittsburgh, Pa Marc G. De Baets, MD, Collina d'Oro, Ticino, Switzerland Kristof De Meerleer, MD, Sint Blasius Boekel-Zwalm, Belgium Seyed A. Emamian, MD, PhD, Rockville, Md Akira Fujikawa, MD, Tokyo, Japan Vidisha V. Ghole, MD, Irving, Tex Michael Griffin, MD, PhD, Milwaukee, Wis Akifumi Hagiwara, MD, Tokyo, Japan Christoph Hefel, Feldkirch, Austria Noriatsu Ichiba, MD, Otsu, Shiga, Japan Takashi Ikeuchi, Moriyama, Shiga, Japan Takao Kiguchi, MD, Niigata, Japan Takuji Kiryu, MD, PhD, Gifu, Japan Mario A. Laguna, MD, Milwaukee, Wis David A. Lisle, MBBS, Brisbane, Queensland, Australia Rafael M. Loureiro, MD, Sao Paulo, Sao Paulo, Brazil Frederick A. Mann, MD, Seattle, Wash Satoshi Matsushima, MD, Tokyo, Japan Claire McArthur, MBChB, FRCR, Glasgow, United Kingdom Kenichi Mizuki, MD, Hamamatsu-shi, Shizuokaken, Japan Jose Mondello, MD, Buenos Aires, Argentina Tomokazu Nishiguchi, MD, PhD, Osaka, Japan Mizuki Nishino, MD, Boston, Mass Roque Oca, MD, Vigo, Pontevedra, Spain Vishal Panchal, San Francisco, Calif David M. Panicek, MD, New York, NY Yeliz Pekcevik, Izmir, Turkey Francesco Pierazzoli, MD, Padua, Italy John M. Plotke, MD, Naperville, Ill Rubem Pochaczevsky, MD, Bronx, NY
Ilias Primetis, MD, Athens, Greece Ryan P. Rebello, MD, Dundas, Ontario, Canada Akihiko Sakata, MD, Kyoto, Japan Meir H. Scheinfeld, MD, Suffern, NY Steven M. Schultz, MD, Fort Worth, Tex Stephen D. Scotti, MD, Edina, Minn Matthew P. Shapiro, MD, Charlottesville, Va Hideki Shima, MD, Narita, Chiba Prefecture, Japan Taro Shimono, MD, Osaka, Japan Ho L. Sie, MD, Henderson, Nev Luis A. Sosa, MD, Milwaukee, Wis Hongliang Sun, MD, Beijing, China Eliko Tanaka, MD, Yokohama, Japan Douglas L. Teich, MD, Brookline, Mass Meric Tuzun, MD, Ankara, Turkey Atsushi Uehara, Niigata, Japan Piet K. Vanhoenacker, MD, Moorsel, Belgium Bonny Varghese, MD, FRANZCR, Melbourne, Victoria, Australia Haruo Watanabe, MD, Gifu, Japan Terry E. Williams, MD, PhD, Louisville, Ky Tatsuya Yamamoto, MD, Yoshida-gun, Fukui, Japan Kurata Yasuhisa, MD, Kobe, Hyogo, Japan Hajime Yokota, MD, Chiba, Japan Rika Yoshida, MD, Utsunomiya, Tochigi, Japan Kaneko You, Gifu, Japan Ahmed Zidan, MD, Barcelona, Spain
Resident group responses CT Scanner Residents Universidad Nacional Autonoma de Mexico, Mexico City, Mexico Mater Dei Hospital Radiology Residents, Malta
The following individuals provided the correct answer for Case 198. David M. Panicek, MD, New York, NY Ayako Tamura, MD, Tokuo, Japan
radiology.rsna.org n Radiology: Volume 270: Number 1—January 2014
Note: This copy is for your personal non-commercial use only. To order presentation-ready copies for distribution to your colleagues or clients, contact us at www.rsna.org/rsnarights.
Diagnosis Please
Case 201: Glomus Tumor of the Breast1
Mona El Khoury, MD Danh Tran-Tranh, MD Donato Terrone, MD Julie David, MD Lucie Lalonde, MD Isabelle Trop, MD
History
A 55-year-old woman presented to our breast clinic with 1-year history of lancinating pain in the right breast that was exacerbated by blunt pressure and cold temperature. She denied any family history of breast cancer or any personal history of trauma, infection, or surgery of the breast. On clinical examination, exquisite pain was generated by simple light pressure over the upper outer quadrant of the right breast. Mammography and ultrasounography (US) with Doppler analysis of the right breast were painful but were performed with the least pressure possible.
Imaging Findings
Part one of this case appeared 4 months previously and may contain larger images. Published online 10.1148/radiol.13120919 Content code: Radiology 2014; 270:302–306 1 From
the Departments of Radiology (M.E.K., D. Terrone, J.D., L.L., I.T.) and Pathology (D. Tran-Tranh), Centre Hospitalier Universitaire de l’Université de Montréal, 3840 Rue Saint Urbain, Montréal, QC, Canada H2W 1T8. Received May 8, 2012; revision requested June 8; revision received August 2; accepted August 3; final version accepted August 22. Address correspondence to M.E.K. (e-mail: elkhourymona @yahoo.fr). Conflicts of interest are listed at the end of this article. q RSNA, 2014
302
Discussion
Bilateral mammograms (Fig 1) revealed an oval hyperdense mass in the upper outer quadrant of the right breast, with partially obscured margins and no microcalcifications. US examination of the right breast (Fig 2a) was extremely painful. Exquisite pain was generated when the probe precisely overlaid an oval, hypoechoic, well-circumscribed mildly lobulated superficially located mass that correlated with the mammographic finding. At color Doppler analysis and spectral assessment, this mass had arterial and venous vessels without shunting and an efferent vessel (Fig 2b, 2c). The patient underwent surgical excision. Pathologic analysis (Fig 3) revealed the presence of a well-defined mass within the fibroadipose tissue characterized by a proliferation of vascular structures surrounded by small monomorphic uniform cells with round nuclei and eosinophilic cytoplasm. The center of the lesion displayed myxoid changes. No substantial mitotic activity or cytologic atypia were noted. At immunohistochemical analy sis, the round uniform cells stained positive for smooth muscle actin and vimentin, weakly positive for CD34, and negative for keratin AE1 and AE3, CD31, and desmin.
The patient’s clinical presentation with chronic pain in the right breast and a pinpoint exquisite tenderness over a superficially located hypervascular mass was unusual but virtually pathognomonic for glomus tumor. The diagnosis was confirmed after surgical excision and pathologic analysis. Glomus tumors are uncommon, extremely painful usually benign mesenchymal neoplasms. They arise from the glomus body, which is a specialized neurovascular organ composed of an afferent arteriole, anastomotic vessel (Sucquet-Hoyer canal), efferent collecting vein, intraglomerular reticulum, and capsular portion. The Sucquet-Hoyer canal is surrounded by endothelial cells and spherical uniform glomus cells thought to be modified smooth muscle cells (1–3). The normal glomus body acts as a contractile neuromyoarterial receptor that regulates peripheral blood flow and is thus involved in temperature and blood pressure regulation. Normal glomus bodies are present in the deepest layer of the dermis (ie, the reticular dermis) throughout the body (2). Histologically, glomus tumors are formed, as a rule, by uniform round
radiology.rsna.org n Radiology: Volume 270: Number 1—January 2014
DIAGNOSIS PLEASE: Glomus Tumor of the Breast
El Khoury et al
Figure 1
Figure 1: (a) Craniocaudal and (b) mediolateral oblique mammograms of the right breast show an oval-shaped hyperdense mass with partially obscured margins in the upper outer quadrant (arrow). Note the mammographically visible nipple markers.
cells with distinct cell borders and a prominent capillary vasculature at the periphery of the mass. They are classified as glomangiomas when their vascular structures are prominent (4), and they may be mistaken for cutaneous round cell tumors or sweat gland tumors when the glomus cells in the tumor are numerous and overshadow the vascular component (5). The high velocity flow within the intratumoral vessels accounts for the high vascularity noted at color Doppler analysis, which is specific for the diagnosis (6). Glomus tumors should be distinguished from chemodectomas (paragangliomas), which may also be designated as glomus tumors or glomus jugular tumors but are different entities that originate from paraganglion cells of the parasympathetic system (5).
Glomus tumors are typically solitary, small, and encountered superficially in the extremities, mainly the subungual areas. They may, however, be atypical, multiple, large, or infiltrative, and they can occur anywhere in the body, including in the gastrointestinal and genitourinary tracts, lung, mediastinum, liver, pancreas, ovary, bone, and soft tissue (1). To our knowledge, only two cases of glomus tumor have been previously reported in the breast (one in a male patient [7] and one in a female patient [8]) and two cases have been reported in the axilla (9,10). When cutaneous, glomus tumors are usually benign, encapsulated, smaller than 1 cm in diameter, and manifest as a blue-red nodule with characteristic paroxysmal pain and sensitivity to cold. This triad of pain, tenderness, and sen-
Radiology: Volume 270: Number 1—January 2014 n radiology.rsna.org
sitivity to cold is known to be pathognomonic of glomus tumors (8) and enables distinction of this rare entity from the other more frequently encountered superficial breast masses, including hemangiomas, angiolipomas, angiosarcomas, arteriovenous malformations, false aneurysms, hemangiopericytomas, and granular cell tumors. Hemangioma is the most frequent benign vascular tumor in the breast. It typically manifests as a hypoechoic well-defined oval mass, and it may be of the cavernous or capillary type depending on the size of its vascular channels (11). It may be hypervascular at color Doppler US, but unlike glomus tumors, it has a more superficial location in the papillary dermis or the epidermis and is not painful (6). Angiolipoma is a fat-containing benign tumor admixed with vascular prolif303
DIAGNOSIS PLEASE: Glomus Tumor of the Breast
El Khoury et al
Figure 2
Figure 2: (a) Radial US image of the right breast shows an oval-shaped well-marginated mildly lobulated and superficially located mass (arrows). (b) Radial US color Doppler image shows internal vascularity with turbulent arterial and venous blood flow and an efferent vessel. (c) Radial US spectral Doppler image shows high velocity arterial flow within the mass.
Figure 3
Figure 3: (a, b) Histopathologic slices stained with hematoxylin-eosin (original magnification, 320 and 3200 in a and b, respectively) show nodular proliferation of small round cells with vascular structures (arrows), which is in keeping with a glomus tumor.
erations and is typically hyperechoic (12). It may be painful to palpation. The pain often diminishes over time, and un304
like in glomus tumors, it is not intensified by thermal changes. The vessels in an angiolipoma are small-caliber capil-
laries, often containing thrombi and surrounded by fat. They do not show any tendency for malignant transformation
radiology.rsna.org n Radiology: Volume 270: Number 1—January 2014
DIAGNOSIS PLEASE: Glomus Tumor of the Breast
or local recurrence when surgically excised (13). Angiosarcoma may be primary or secondary, associated with previous radiation therapy or lymphedema, and may show, as glomus tumors, a blue skin discoloration and high vascularity on Doppler US images; however, it manifests as an ill-defined mass with skin thickening (12,14). Thus, it is not the most likely diagnosis in this patient given the clinical and imaging findings of a well-defined mass. Hemangiopericytoma is a benign vascular tumor with a potentially malignant nature originating from the capillary pericytes, which are the contractile cells surrounding the walls of the vessels. Hemangiopericytoma can be hypervascular on color Doppler images, with a feeding vessel (15,16). False aneurysm or communicating hematoma surrounded by the adventitia of an arterial vessel and perivascular tis sue may manifest as an anechoic ovoid mass with turbulent flow on color Doppler images; however, it displays the typical yin-yang appearance and occurs in the setting of acute trauma and is often pulsatile (12). Granular cell tumors arise from Schwann cells of peripheral nerves. About 5%–8% of these tumors occur in the breast, usually from the dermis, and project into subcutaneous tissue. They may manifest as a mass with either well-circumscribed or spiculated and infiltrative margins usually associated with overlying skin thickening or retraction (14,17). Amid all these superficially located breast masses with different degree of vascularity, only glomus tumors are associated with exquisite pain. Very rare cases of malignant glomus tumors have been reported. Folpe et al (18) reviewed 52 unusual cases of glomus tumors and classified them as (a) malignant, with high risk of metastasis when deeply located and larger than 2 cm or when displaying atypical mitotic figures or moderate to high nuclear grade and more than five mitotic figures per 50 high-power fields; (b) symplastic when displaying a high nuclear grade and no other malignant feature; (c) of
El Khoury et al
uncertain malignant potential when lacking firm criteria for malignancy or benignancy, and (d) glomangiomatosis when they have the histologic features of diffuse angiomatosis and excess glomus cells. Immunohistochemical analysis helps establish the correct diagnosis, since glomus tumors stain negative for desmin, cytokeratin, and S100 and positive for vimentin and smooth muscle actin (18). Unlike glomus tumors, melanomas, Merckel cell carcinomas, and eccrine spireadenomas express S100 and cytokeratin, whereas the extremely rare primitive neuroectodermal tumors and neuroblastomas do not express muscle actin (18). When a glomus tumor is suspected in the breast, US is the first imaging technique preformed because patients usually are unable to tolerate compression because of severe pain (6). At imaging, glomus tumors typically manifest as an extremely painful, hypervascular well-marginated superficial mass (6). The treatment of choice is surgical excision (6). If inadequately excised, glomus tumors—particularly subungual ones, may recur in 1%–2% of cases (2,7). In summary, glomus tumors that develop outside the more typical subungual location, including those that develop in the breasts, are uncommon and present a diagnostic challenge. The most important initial step for making the correct diagnosis is clinical suspicion in a patient who presents with chronic local pain, dysesthesia, and pinpoint tenderness to pressure anywhere in the body, including the breast, as in this patient. By incorporating the clinical presentation with imaging findings (mainly US findings), the breast radiologist can effectively establish the diagnosis with no need for percutaneous biopsy, which is generally intolerable due to extreme pain. Disclosures of Conflicts of Interest: M.E.K. No relevant conflicts of interest to disclose. D. Tran-Thanh No relevant conflicts of interest to disclose. D. Terrone No relevant conflicts of interest to disclose. J.D. No relevant conflicts of interest to disclose. L.L. No relevant conflicts
Radiology: Volume 270: Number 1—January 2014 n radiology.rsna.org
of interest to disclose. I.T. No relevant conflicts of interest to disclose.
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14. Kalli S, Freer PE, Rafferty EA. Lesions of the skin and superficial tissue at breast MR imaging. RadioGraphics 2010;30(7):1891–1913. 15. van Kints MJ, Tham RT, Klinkhamer PJ, van den Bosch HC. Hemangiopericytoma of the breast: mammographic and sonographic findings. AJR Am J Roentgenol 1994;163(1):61–63. 16. Vilanova JC, Barceló J, Smirniotopoulos JG, et al. Hemangioma from head to toe: MR imaging with pathologic correlation. RadioGraphics 2004;24(2):367–385. 17. Giess CS, Raza S, Birdwell RL. Distinguishing breast skin lesions from superficial breast parenchymal lesions: diagnostic criteria, imaging characteristics, and pitfalls. RadioGraphics 2011;31(7):1959–1972. 18. Folpe AL, Fanburg-Smith JC, Miettinen M, Weiss SW. Atypical and malignant glomus tumors: analysis of 52 cases, with a proposal for the reclassification of glomus tumors. Am J Surg Pathol 2001;25(1):1–12.
Congratulations to the 63 individuals and two resident groups that submitted the most likely diagnosis (glomus tumor of the breast) for Diagnosis Please, Case 201. The names and locations of the individuals and resident groups, as submitted, are as follows:
Individual responses Albert J. Alter, MD, PhD, Blanchardville, Wis Sebastian Bravo-Grau, MD, MSc, Santiago, Region Metropolitana, Chile Ian A. Burgess, MD, North Sydney, New South Wales, Australia Michael H. Childress, MD, Silver Spring, Md
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Christopher Chu, MBBS, FRANZC, Sydney, New South Wales, Australia Theresa M. Corrigan, MD, Louisville, Ky Jezreel C. Da Costa, Porto Velho, Brazil Anil K. Dasyam, MD, Pittsburgh, Pa Marc G. De Baets, MD, Collina d'Oro, Ticino, Switzerland Kristof De Meerleer, MD, Sint Blasius Boekel-Zwalm, Belgium Seyed A. Emamian, MD, PhD, Rockville, Md Akira Fujikawa, MD, Tokyo, Japan Vidisha V. Ghole, MD, Irving, Tex Michael Griffin, MD, PhD, Milwaukee, Wis Akifumi Hagiwara, MD, Tokyo, Japan Christoph Hefel, Feldkirch, Austria Noriatsu Ichiba, MD, Otsu, Shiga, Japan Takashi Ikeuchi, Moriyama, Shiga, Japan Takao Kiguchi, MD, Niigata, Japan Takuji Kiryu, MD, PhD, Gifu, Japan Mario A. Laguna, MD, Milwaukee, Wis David A. Lisle, MBBS, Brisbane, Queensland, Australia Rafael M. Loureiro, MD, Sao Paulo, Sao Paulo, Brazil Frederick A. Mann, MD, Seattle, Wash Satoshi Matsushima, MD, Tokyo, Japan Claire McArthur, MBChB, FRCR, Glasgow, United Kingdom Kenichi Mizuki, MD, Hamamatsu-shi, Shizuokaken, Japan Jose Mondello, MD, Buenos Aires, Argentina Tomokazu Nishiguchi, MD, PhD, Osaka, Japan Mizuki Nishino, MD, Boston, Mass Roque Oca, MD, Vigo, Pontevedra, Spain Vishal Panchal, San Francisco, Calif David M. Panicek, MD, New York, NY Yeliz Pekcevik, Izmir, Turkey Francesco Pierazzoli, MD, Padua, Italy John M. Plotke, MD, Naperville, Ill Rubem Pochaczevsky, MD, Bronx, NY
Ilias Primetis, MD, Athens, Greece Ryan P. Rebello, MD, Dundas, Ontario, Canada Akihiko Sakata, MD, Kyoto, Japan Meir H. Scheinfeld, MD, Suffern, NY Steven M. Schultz, MD, Fort Worth, Tex Stephen D. Scotti, MD, Edina, Minn Matthew P. Shapiro, MD, Charlottesville, Va Hideki Shima, MD, Narita, Chiba Prefecture, Japan Taro Shimono, MD, Osaka, Japan Ho L. Sie, MD, Henderson, Nev Luis A. Sosa, MD, Milwaukee, Wis Hongliang Sun, MD, Beijing, China Eliko Tanaka, MD, Yokohama, Japan Douglas L. Teich, MD, Brookline, Mass Meric Tuzun, MD, Ankara, Turkey Atsushi Uehara, Niigata, Japan Piet K. Vanhoenacker, MD, Moorsel, Belgium Bonny Varghese, MD, FRANZCR, Melbourne, Victoria, Australia Haruo Watanabe, MD, Gifu, Japan Terry E. Williams, MD, PhD, Louisville, Ky Tatsuya Yamamoto, MD, Yoshida-gun, Fukui, Japan Kurata Yasuhisa, MD, Kobe, Hyogo, Japan Hajime Yokota, MD, Chiba, Japan Rika Yoshida, MD, Utsunomiya, Tochigi, Japan Kaneko You, Gifu, Japan Ahmed Zidan, MD, Barcelona, Spain
Resident group responses CT Scanner Residents Universidad Nacional Autonoma de Mexico, Mexico City, Mexico Mater Dei Hospital Radiology Residents, Malta
The following individuals provided the correct answer for Case 198. David M. Panicek, MD, New York, NY Ayako Tamura, MD, Tokuo, Japan
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