Correspondence
Metastasizing Aggressive Angiomyxoma
N Engl J Med 1999; 341:1772December 2, 1999
- Article
To the Editor:
Aggressive angiomyxoma is a rare myofibroblastic tumor that arises mainly in the perineum and genitalia in premenopausal women. Histologically, the tumor has a monomorphic pattern, with small, spindle-shaped mesenchymal cells dispersed in a loose, myxoid matrix and a characteristic vascular component. Cellularity is generally low, and mitoses are not seen. Despite this innocuous-looking histologic pattern, angiomyxoma has a strong tendency to be locally aggressive, with infiltrative growth and local recurrence.1 In approximately 100 documented cases, however, no evidence of distant metastasis has been reported. We describe a case of aggressive angiomyxoma of the pelvis that metastasized to the mediastinum and lungs.
A 63-year-old woman presented with a seven-week history of nonspecific abdominal symptoms. Abdominal computed tomography (CT) showed four pelvic tumors, up to 9.5 by 7 cm, with many enlarged lymph nodes adjacent to the iliac vessels and aorta. Thoracic CT showed a mass, 6 by 10 cm, in the left upper lobe, multiple round lesions up to 15 mm in diameter in both lungs, and many enlarged mediastinal lymph nodes. Histologic examination of a specimen from a CT-guided, core needle punch biopsy of a pelvic tumor revealed a mesenchymal tumor.
During exploratory laparotomy, a myxoid tumor was found in the pelvis, with teardrop-shaped metastases in the greater omentum, the mesentery, and the peritoneum. All visible tumor was removed, and a radical resection performed.
Histologic examination of the specimen showed a myxoid tumor with sparse infiltrates of polymorphic cells clustering around blood vessels (Figure 1AFigure 1
Specimens from the Primary Tumor in the Pelvic Soft Tissue (Panel A) and the Metastatic Pulmonary Lesion (Panel B) (Hematoxylin and Eosin, ×120).). Immunohistochemical studies showed strong staining for CD34, estrogen receptors, and progesterone receptors. Staining for actin, desmin, and vimentin was intermediate, and staining for S-100 protein was negative. The histologic diagnosis was aggressive angiomyxoma. A core needle–biopsy specimen of one of the intrathoracic lesions showed the same histologic pattern (Figure 1B), confirming the metastatic spread of the primary tumor.Aggressive angiomyxoma was first described in 1983.2 Despite morphologic and histologic indicators of malignancy, to our knowledge there have been no reports of the metastatic spread of such tumors. In the present case, the tumor had not only spread diffusely throughout the abdominal cavity but had also caused multiple metastases in the mediastinum and both lungs. Other diagnoses, such as myxoid liposarcoma and angiomyofibroblastoma,3 could be clearly ruled out on the basis of histomorphologic and immunohistochemical criteria.4 These findings suggest that aggressive angiomyxoma can no longer be considered a purely localized disease.
4 ReferencesRamin Michael Siassi, M.D.
Thomas Papadopoulos, M.D., Ph.D.
Klaus E. Matzel, M.D., Ph.D.
University of Erlangen, 91054 Erlangen, Germany1
Begin LR ,Clement PB ,Kirk ME ,Jothy S ,McCaughey WT ,Ferenczy A . Aggressive angiomyxoma of pelvic soft parts: a clinicopathologic study of nine cases. Hum Pathol 1985;166:621-628
CrossRef | Web of Science2
Steeper TA ,Rosai J . Aggressive angiomyxoma of the female pelvis and perineum: report of nine cases of a distinctive type of gynecologic and soft-tissue neoplasm. Am J Surg Pathol 1983;7:463-475
CrossRef | Web of Science | Medline3
Granter SR ,Nucci MR ,Fletcher CD . Aggressive angiomyxoma: reappraisal of its relationship to angiomyofibroblastoma in a series of 16 cases. Histopathology 1997;30:3-10
CrossRef | Web of Science | Medline4
Brooks SE ,Balidimos I ,Reuter KL ,Khan A . Aggressive angiomyxoma of the vulva: impact of GnRH agonists. Medscape Womens Health 1998;3:4-4
Medline
- Citing Articles (42)
Citing Articles
1
Brian J. Sutton, Jennifer Laudadio. (2012) Aggressive Angiomyxoma. Archives of Pathology & Laboratory Medicine 136:2, 217-221
CrossRef2
Junzu Geng, Bofeng Cao, Liping Wang. (2012) Aggressive Angiomyxoma: an Unusual Presentation. Korean Journal of Radiology 13:1, 90
CrossRef3
Virginia Oliva Shaffer, Mariana Berho, Lester Rosen. (2011) A Case of Intraluminal Rectal Aggressive Angiomyxoma. Colorectal Diseaseno-no
CrossRef4
Yaku Tanaka, Hiromasa Hori, Itsuo Gorai. (2011) Aggressive Angiomyxoma in the Pelvis. Journal of Lower Genital Tract Disease 15:3, 254-256
CrossRef5
Krishna Dahiya, Shaveta Jain, Nirmala Duhan, Smiti Nanda, Parveen Kundu. (2011) Aggressive angiomyxoma of vulva and vagina: a series of three cases and review of literature. Archives of Gynecology and Obstetrics 283:5, 1145-1148
CrossRef6
Ping Xiao, Dawei Liu, Jianjun Peng, Zhenhua Gao, Yu Dong, Huiqun Wu, Anjia Han. (2011) Aggressive angiomyxoma of the sphenoidal sinus. Pathology 43:1, 79-81
CrossRef7
Yassir Ait-Benkaddour, Abderrahim Aboulfalah, Badiaa Belaabidia, Hassan Abbassi. (2010) Aggressive Vulvar Angiomyxoma: Report of Two Cases and Review of the Literature. Journal of Gynecologic Surgery 26:3, 205-206
CrossRef8
Santiago Mera Velasco, Antonio J. Cabello Burgos, Manuel Ruiz López, Iván González Poveda, Julio Santoyo Santoyo. (2010) Resección laparoscópica de angiomixoma pélvico agresivo. Cirugía Española 88:2, 121-122
CrossRef9
W. Glenn McCluggage, Lynnette Connolly, Hilary A. McBride. (2010) HMGA2 is a Sensitive But Not Specific Immunohistochemical Marker of Vulvovaginal Aggressive Angiomyxoma. The American Journal of Surgical Pathology 34:7, 1037-1042
CrossRef10
D C Sylvester, S Kortequee, J W Moor, C J Woodhead, K A Maclennan. (2010) Aggressive angiomyxoma of larynx: case report and literature review. The Journal of Laryngology & Otology 124:07, 793-795
CrossRef11
Hyun-Soo Kim, Gou Young Kim, Sung-Jig Lim, Kyung-Do Ki, Hyun Cheol Kim. (2010) Giant superficial angiomyxoma of the vulva: a case report and review of the literature. Journal of Cutaneous Pathology 37:6, 672-677
CrossRef12
A. Plumb, J.H. Shanks, R. Kochhar. (2010) Development of aggressive angiomyxoma like tumour in a renal transplant. Clinical Radiology 65:5, 423-426
CrossRef13
K. Haldar, I.E. Martinek, S. Kehoe. (2010) Aggressive angiomyxoma: A case series and literature review. European Journal of Surgical Oncology (EJSO) 36:4, 335-339
CrossRef14
Zhang Jingping, Zhu Chunfu. (2010) Clinical Experiences on Aggressive Angiomyxoma in China (Report of 93 Cases). International Journal of Gynecological Cancer 20:2, 303-307
CrossRef15
Mehmet Coskun Salman, Gamze Mocan Kuzey, Nasuh Utku Dogan, Kunter Yuce. (2009) Aggressive angiomyxoma of vulva recurring 8 years after initial diagnosis. Archives of Gynecology and Obstetrics 280:3, 485-487
CrossRef16
Tomonori Minagawa, Keiji Matsushita, Ryo Shimada, Hiroto Takayama, Risako Hiraga, Takeshi Uehara, Yasushi Murata. (2009) Aggressive angiomyxoma mimicking inguinal hernia in a man. International Journal of Clinical Oncology 14:4, 365-368
CrossRef17
Ning-xia SUN, Yue DAI, Wen LI. (2009) Angiomyxoma invading the vulva: a report of 2 cases. Academic Journal of Second Military Medical University 29:3, 346-347
CrossRef18
W G McCluggage. (2009) Recent developments in vulvovaginal pathology. Histopathology 54:2, 156-173
CrossRef19
Danya Sereda, Philippe Sauthier, Rachid Hadjeres, Deana Funaro. (2009) Aggressive Angiomyxoma of the Vulva. Journal of Lower Genital Tract Disease 13:1, 46-50
CrossRef20
E. J. Heffernan, M. M. Hayes, F. O. Alkubaidan, P. W. Clarkson, P. L. Munk. (2008) Aggressive angiomyxoma of the thigh. Skeletal Radiology 37:7, 673-678
CrossRef21
Chien-Yu Pai, Shin Nieh, Jih-Chin Lee, Chung-Ping Lo, Herng-Sheng Lee. (2008) Aggressive angiomyxoma of supraclavicular fossa: A case report. Head & Neck 30:6, 821-824
CrossRef22
Neil J. Rawlinson, William W. West, Marilu Nelson, Julia A. Bridge. (2008) Aggressive angiomyxoma with t(12;21) and HMGA2 rearrangement: report of a case and review of the literature. Cancer Genetics and Cytogenetics 181:2, 119-124
CrossRef23
U. P. Umeadi, A. S. Ahmed, B. Winter-Roach, J. Murphy, P. Shenjere, R. J. Slade. (2008) Aggressive angiomyxoma of the vulva and perineum: A case report. Journal of Obstetrics & Gynaecology 28:7, 757-759
CrossRef24
Fabiola Medeiros, Michele R. Erickson-Johnson, Gary L. Keeney, Amy C. Clayton, Antonio G. Nascimento, Xiaoke Wang, Andre M. Oliveira. (2007) Frequency and characterization ofHMGA2 andHMGA1 rearrangements in mesenchymal tumors of the lower genital tract. Genes, Chromosomes and Cancer 46:11, 981-990
CrossRef25
Takahiro Tsuji, Mitsuhiro Yoshinaga, Yukiko Inomoto, Shuuhei Taguchi, Tsutomu Douchi. (2007) Aggressive Angiomyxoma of the Vulva With a Sole t(5;8)(p15;q22) Chromosome Change. International Journal of Gynecological Pathology 26:4, 494-496
CrossRef26
Chia-Chang Wu, Stephen Shei-Dei Yang, Daniel T. H. Chin, Cheng-Hsing Hsieh, Yu-Mei Hsueh, Yao-Chou Tsai. (2007) Scrotal aggressive angiomyxoma mimicking inguinal hernia. Asian Journal of Andrology 9:5, 723-725
CrossRef27
Eric J. Heffernan, Fahad O. Alkubaidan, Paul W. Clarkson, Peter L. Munk. (2007) Unusual location of aggressive angiomyxoma in a male. European Journal of Radiology Extra 63:1, 39-42
CrossRef28
I.J.M. Han-Geurts, A.N. van Geel, L. van Doorn, M. den Bakker, A.M.M. Eggermont, C. Verhoef. (2006) Aggressive angiomyxoma: multimodality treatments can avoid mutilating surgery. European Journal of Surgical Oncology (EJSO) 32:10, 1217-1221
CrossRef29
N Mourra, C Hoeffel, S Gaujoux, J-M Coindre, J-F Flejou. (2006) Myxoid perineal tumour in a 25-year-old woman. Histopathology 49:5, 533-536
CrossRef30
A. Wiser, J. Korach, W. H. Gotlieb, E. Fridman, S. Apter, G. Ben-Baruch. (2006) Importance of accurate preoperative diagnosis in the management of aggressive angiomyxoma: report of three cases and review of the literature. Abdominal Imaging 31:3, 383-386
CrossRef31
Francesca Micci, Ioannis Panagopoulos, Bodil Bjerkehagen, Sverre Heim. (2006) Deregulation of HMGA2 in an aggressive angiomyxoma with t(11;12)(q23;q15). Virchows Archiv 448:6, 838-842
CrossRef32
Todd Boren, James Lapolla, Martin Martino, Pranita Nirgudkar. (2006) Aggressive Angiomyxoma. Journal of Pelvic Medicine and Surgery 12:3, 151-156
CrossRef33
BENJAMIN K. CANALES, DEREK WEILAND, NATHAN HOFFMAN, JOEL SLATON, MICHAEL TRAN, J. CARLOS MANIVEL, MANOJ MONGA. (2006) Angiomyofibroblastoma-like tumors (cellular angiofibroma). International Journal of Urology 13:2, 177-179
CrossRef34
J. F. Graadt Roggen, J. A. M. Unnik, I. H. Briaire-de Bruijn, P. C. W. Hogendoorn. (2005) Aggressive angiomyxoma: a clinicopathological and immunohistochemical study of 11 cases with long-term follow-up. Virchows Archiv 446:2, 157-163
CrossRef35
Bonnie L. Balzer, Teri A. Longacre. (2005) Aggressive Angiomyxoma of the Female Genital Tract. Pathology Case Reviews 10:1, 46-54
CrossRef36
Lavanya RAI, Varalaxmi NANDYALA, Jyothi SHETTY, Vijay KUMAR, Laxmy RAO. (2004) Aggressive angiomyxoma: An important differential diagnosis for a vaginal mass. The Australian and New Zealand Journal of Obstetrics and Gynaecology 44:4, 367-368
CrossRef37
YOSHITOMO CHIHARA, KIYOHIDE FUJIMOTO, SATOSHI TAKADA, AKIHIDE HIRAYAMA, MASAKI CHO, KATSUNORI YOSHIDA, SEIICHIRO OZONO, YOSHIHIKO HIRAO. (2003) Aggressive angiomyxoma in the scrotum expressing androgen and progesterone receptors. International Journal of Urology 10:12, 672-675
CrossRef38
Stella Blandamura, Julia Cruz, Lourdes Faure Vergara, Isidro Machado Puerto, Vito Ninfo. (2003) Aggressive angiomyxoma: a second case of metastasis with patient’s death. Human Pathology 34:10, 1072-1074
CrossRef39
Shakira Payne, Regina Adair, Jesus Alvarez, Debra Heller, Anthony Caggiano. (2003) Aggressive Angiomyxoma of the Space of Retzius: A Case Report. Journal of Lower Genital Tract Disease 7:4, 304-306
CrossRef40
M. Joyce, D. C. Winter, M. Leader, A. Reilly, J. M. Deasy. (2001) Aggressive angiomyxoma of the perineum. Irish Journal of Medical Science 170:4, 265-266
CrossRef41
Margarita De La Ossa, Amilcar Castellano-Sanchez, Elena Alvarez, William Smoak, Morton J. Robinson. (2001) Sonographic appearance of aggressive angiomyxoma of the scrotum. Journal of Clinical Ultrasound 29:8, 476-478
CrossRef42
G. Petur Nielsen, Robert H. Young. (2001) Mesenchymal Tumors and Tumor-Like Lesions of the Female Genital Tract: A Selective Review with Emphasis on Recently Described Entities. International Journal of Gynecological Pathology 20:2, 105-127
CrossRef
