Warning: mkdir(): Permission denied in /home/virtual/lib/view_data.php on line 81

Warning: fopen(upload/ip_log/ip_log_2024-03.txt): failed to open stream: No such file or directory in /home/virtual/lib/view_data.php on line 83

Warning: fwrite() expects parameter 1 to be resource, boolean given in /home/virtual/lib/view_data.php on line 84
Cotyledonoid Dissecting Leiomyoma of the Uterus with Intravascular Luminal Growth: A Case Study
Skip Navigation
Skip to contents

J Pathol Transl Med : Journal of Pathology and Translational Medicine

OPEN ACCESS
SEARCH
Search

Articles

Page Path
HOME > J Pathol Transl Med > Volume 47(5); 2013 > Article
Case Study
Cotyledonoid Dissecting Leiomyoma of the Uterus with Intravascular Luminal Growth: A Case Study
Na Rae Kim, Chan Yong Park1, Hyun Yee Cho
Korean Journal of Pathology 2013;47(5):477-480.
DOI: https://doi.org/10.4132/KoreanJPathol.2013.47.5.477
Published online: October 25, 2013

Department of Pathology, Gachon University Gil Medical Center, Incheon, Korea.

1Department of Gynecology and Obstetrics, Gachon University Gil Medical Center, Incheon, Korea.

Corresponding Author: Hyun Yee Cho, M.D. Department of Pathology, Gachon University Gil Medical Center, 21 Namdong-daero 774beon-gil, Namdong-gu, Incheon 405-760, Korea. Tel: +82-32-460-3865, Fax: +82-32-460-3073, 'hicho@gilhospital.com'
• Received: November 12, 2012   • Revised: December 24, 2012   • Accepted: January 3, 2013

© 2013 The Korean Society of Pathologists/The Korean Society for Cytopathology

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

  • 10,180 Views
  • 67 Download
  • 5 Crossref
  • 6 Scopus
  • Here, we report the case of a 43-year-old female who was diagnosed with a cotyledonoid dissecting leiomyoma (CDL) of the uterus. CDL is a recently described and extremely rare variant of a benign leiomyoma that can grossly masquerade as a malignancy. The 13-cm sized tumor was located primarily on the extrauterine surface as an intrauterine continuity, which showed dark red, congested, bulbous protuberances. It was multinodular appearance, encasing the bilateral adnexae and the left iliac vein. Microscopically, the nodules were separated by extensive hydropic degeneration. The nodules were composed of cigar-shaped spindle cells with no mitosis, cellular pleomorphism or coagulation necrosis. They also showed an intravascular luminal growth pattern. CDL with intravascular growth was diagnosed after excluding intravascular leiomyomatosis, disseminated peritoneal leiomyomatosis, and benign metastasizing leiomyoma. The present case is the second reported case of CDL in Korea. Recognition of this rare and bizarre, malignancy-mimicking leiomyoma is crucial to prevent inappropriate treatment.
Cotyledonoid dissecting leiomyoma (CDL), also known as a Sternberg tumor or "grape-like" leiomyoma, is an extremely rare variant that mimics malignant gross features but has a benign histology and biological behavior.1 The name "cotyledonoid" leiomyoma originated from the characteristic gross morphology, which includes extrauterine dark red, spongy, bulbous protuberances, also resembling a placenta.2 To date, 41 cases of CDLs have been reported in the English literature.1-9 Here, we report an additional case of CDL. To the best of our knowledge, the present case is the second reported case of CDL in Korea.3 Our case also showed intravascular luminal growth, which is very uncommon in CDLs of the uterus.
Here, we emphasize that clinicians should be aware of this rare entity to prevent unnecessary aggressive treatment due to its warning gross morphology.
A 43-year-old Korean female (gravida 2, para 2) with a three-year history of a palpable abdominal mass was admitted to the gynecology outpatient department. She had a history of two caesarian sections. The patient had been followed up for 12 months. A recent evaluation showed a marked increase in tumor size (up to approximately 8.0 cm) and the presence of abdominal pain. Abdominopelvic computed tomography showed a large enhancing mass measuring 13.0 cm that encased the left adnexa, the uterus, and the left iliac vein (Fig. 1). Omental haziness and small seeding nodules suggested the possibility of seeding carcinomatosis peritonei. Intraoperatively, no collection of pelvic cavity fluid was found. Total abdominal hysterectomy and bilateral salpingo-oophorectomy were performed with removal of the parametrially extended tumor. Grossly, the multinodular mass was dark red, congested and spongy with bulbous protuberances over the extrauterine surface and bilateral broad ligaments (Fig. 2A). Contrary to the dark red color of the external surface, the cut surface showed a homogeneous, grayish tan-colored, multinodular, firm trabeculated appearance (Fig. 2B). No intravascular growth or invasion was identified on gross examination. Leiomyosarcoma was initially suspected. Microscopically, the excised mass was composed of variably sized multiple nodules, which were surrounded by perinoudular widened hydropic degeneration (Fig. 3A). The nodules consisted of fascicles of spindle cells with no cellular pleomorphism, mitotic activity, or coagulation necrosis (Fig. 3B, C). The mass encased the bilateral ovaries and salpinges, which were free from tumor invasion. Intravascular protruding growth was also found (Fig. 3D). Immunostaining for CD34 highlighted the endothelial cells of the blood vessels surrounding the foci of the intravascular growth of the leiomyoma (Fig. 3E). The spindle cells were positive for muscle-specific actin (IA4, prediluted, Dako, Glostrup, Denmark) and desmin (D33, prediluted, Dako). They were negative for CD10 (56C6, prediluted, Dako). CDL with an intravascular growth pattern was diagnosed.
CDL, the least variant of benign uterine leiomyomas, is characterized by extrauterine bulbous expansile growth continuous to the intrauterine location of the mass, mimicking a grape-like appearance.1-3 Due to its bizarre gross morphology (including a large size, widespread invasive growth, etc.) and status as a rarely encountered, unfamiliar variant, an intraoperative impression of malignant neoplasm, especially leiomyosarcoma, was initially suspected. However, uterine CDL can be distinguished from leiomyosarcoma in that the former has a consistency that is not soft but firm instead. In addition, macroscopic differential diagnoses, such as sarcomas (e.g. leiomyosarcomas), can be easily excluded after microscopic observation.1,4 Microscopic differential diagnoses included intravascular leiomyomatosis, disseminated peritoneal leiomyomatosis, and benign metastasizing leiomyoma. Intravascular leiomyomatosis is characterized by a grossly visible vascular invasion of a histologically proven benign smooth muscle tumor.10 Disseminated peritoneal leiomyomatosis shows numerous small (usually less than 2 cm in size) smooth muscle tumorlets spread across the peritoneum.11 Benign metastasizing leiomyoma is a morphologically benign leiomyoma occurring at distant extrauterine sites, such as the lung or regional lymph nodes.12
The exact nature of CDL remains unclear. The exophytic dark red portion of the CDL may be caused by venous obstruction and congestion, and the endophytic intramyometrial portion with poorly defined borders may arise from the parent leiomyoma between the myometrium.2
Ultrasonographic findings are not distinct from other conventional leiomyomas. Magnetic resonance images of CDLs show heterogeneous intensity with patchy isosignal nodules and stromal components with a high signal on T2-weighted images.5 The high signal intensity reflects perinodular hydropic degeneration.6
After reviewing the 42 previously reported cases of CDLs (including the present one), all the cases showed no recurrence or metastases during the follow-up period.1 Clinically, most cases occur in women of reproductive age, ranging from 23 to 73 years (mean, 44 years). Grossly, CDLs are composed of an exophytic subserosal mass and an intramural endophytic portion with continuity. The size of the reported CDLs ranged from 4 to 41 cm (mean, 14.2 cm). Microscopically, the nodules are separated by a fibrovascular connective tissue with marked hydropic changes or congestion filled with numerous vascular tissues.2 The spindle cells show no cellular atypia, mitosis or coagulation necrosis with a dissecting growth pattern due to perinodular extensive hydropic changes.1,2 Intravascular growth is rare, and only 9 cases (21.4%) have been reported in the 42 published cases of CDLs, which includes the present case.1,4,7-9 Foci of intravascular luminal growth pattern may cause misdiagnosis of the condition as a malignancy when present. Intravenous leiomyomatosis behaves in a benign fashion although vascular invasion may extend as far as the extrauterine pelvic veins, the inferior vena cava, and even up to the right side of the heart.10 Similarly, CDLs with intravascular luminal growth have been known to pursue a benign nature but with uncertain biological behavior.7 Further accumulated cases will be required to establish the prognostic impact of intravascular luminal growth in these rare entities.
To avoid misdiagnosis, especially in intraoperative frozen diagnosis, acknowledgment of this rare entity is important because it is known to have a benign biological behavior that mimics the gross morphology of a malignant tumor.

No potential conflict of interest relevant to this article was reported.

  • 1. Smith CC, Gold MA, Wile G, Fadare O. Cotyledonoid dissecting leiomyoma of the uterus: a review of clinical, pathological, and radiological features. Int J Surg Pathol 2012; 20: 330–341. PMID: 22710314. ArticlePubMed
  • 2. Roth LM, Reed RJ, Sternberg WH. Cotyledonoid dissecting leiomyoma of the uterus: the Sternberg tumor. Am J Surg Pathol 1996; 20: 1455–1461. PMID: 8944038. ArticlePubMed
  • 3. Kim MJ, Park YK, Cho JH. Cotyledonoid dissecting leiomyoma of the uterus: a case report and review of the literature. J Korean Med Sci 2002; 17: 840–844. PMID: 12483013. ArticlePubMedPMC
  • 4. Gezginç K, Yazici F, Selimoğlu R, Tavli L. Cotyledonoid dissecting leiomyoma of the uterus with intravascular growth in postmenopausal woman: a case presentation. Int J Clin Oncol 2011; 16: 701–704. PMID: 21331771. ArticlePubMed
  • 5. Preda L, Rizzo S, Gorone MS, Fasani R, Maggioni A, Bellomi M. MRI features of cotyledonoid dissecting leiomyoma of the uterus. Tumori 2009; 95: 532–534. PMID: 19856670. ArticlePubMedPDF
  • 6. Motoshima S, Irie H, Nakazono T, Kamura T, Kudo S. Diffusion-weighted MR imaging in gynecologic cancers. J Gynecol Oncol 2011; 22: 275–287. PMID: 22247805. ArticlePubMedPMC
  • 7. Shelekhova KV, Kazakov DV, Michal M. Cotyledonoid dissecting leiomyoma of the uterus with intravascular growth: report of two cases. Virchows Arch 2007; 450: 119–121. PMID: 17123108. ArticlePubMed
  • 8. Jordan LB, Al-Nafussi A, Beattie G. Cotyledonoid hydropic intravenous leiomyomatosis: a new variant leiomyoma. Histopathology 2002; 40: 245–252. PMID: 11895490. ArticlePubMed
  • 9. Fukunaga M, Suzuki K, Hiruta N. Cotyledonoid dissecting leiomyoma of the uterus: a report of four cases. APMIS 2010; 118: 331–333. PMID: 20402679. ArticlePubMed
  • 10. Lo KW, Lau TK. Intracardiac leiomyomatosis: case report and literature review. Arch Gynecol Obstet 2001; 264: 209–210. PMID: 11205711. ArticlePubMed
  • 11. Quade BJ, McLachlin CM, Soto-Wright V, Zuckerman J, Mutter GL, Morton CC. Disseminated peritoneal leiomyomatosis: clonality analysis by X chromosome inactivation and cytogenetics of a clinically benign smooth muscle proliferation. Am J Pathol 1997; 150: 2153–2166. PMID: 9176406. PubMedPMC
  • 12. Nuovo GJ, Schmittgen TD. Benign metastasizing leiomyoma of the lung: clinicopathologic, immunohistochemical, and micro-RNA analyses. Diagn Mol Pathol 2008; 17: 145–150. PMID: 18382364. ArticlePubMedPMC
Fig. 1
Radiologic finding. Abdominopelvic computed tomography reveals a huge pelvic mass encasing the ovaries and salpinges as well as the iliac vein (white arrows) on the left side. Black arrows indicate the iliac arteries.
kjpathol-47-477-g001.jpg
Fig. 2
Gross finding. (A) The resected uterus shows an exophytic bosselated mass with a dark red and firm appearance. Note the multinodular exophytic part (arrow) of the mass extending into the broad ligament. (B) The cut surface is a mass of rubbery reddish and solid multiple nodules, measuring from 0.4 to 3.0 cm in their greatest dimension.
kjpathol-47-477-g002.jpg
Fig. 3
Microscopic finding. (A) Multiple nodules are dissected at the periphery with hydrophic degeneration. (B, C) The perinodular stroma is filled with many congested vessels (B). Interlacing fascicles consist of the spindle cells without cellular atypia, necrosis or mitoses (C). (D, E) The neoplastic leiomyomatous growth into the lumen of the vessel is shown (D). Immunostaining for CD34 reveals vascular lining cells around the intraluminal growth of the cotyledonoid dissecting leiomyoma (E).
kjpathol-47-477-g003.jpg

Figure & Data

References

    Citations

    Citations to this article as recorded by  
    • Cotyledonoid dissecting leiomyoma of the uterus: a case report and review of the literature
      Mahboobeh Chahkandi, Marzieh Ataei, Amir Reza Bina, Farnaz Mozayani, Ali Fanoodi
      Journal of Medical Case Reports.2023;[Epub]     CrossRef
    • Cotyledonoid Leiomyoma Clinical Characteristics, Imaging Features, and Review of the Literature
      Francesca Buonomo, Sofia Bussolaro, Giorgio Giorda, Federico Romano, Stefania Biffi, Giuseppe Ricci
      Journal of Ultrasound in Medicine.2021; 40(7): 1459.     CrossRef
    • The Management of the Cotyledonoid Leiomyoma of the Uterus: A Narrative Review of the Literature
      Francesca Buonomo, Sofia Bussolaro, Clarice de Almeida Fiorillo, Giorgio Giorda, Federico Romano, Stefania Biffi, Giuseppe Ricci
      International Journal of Environmental Research and Public Health.2021; 18(16): 8521.     CrossRef
    • Cotyledonoid dissecting leiomyoma of the uterus: A report of four cases and a review of the literature
      TIANMIN XU, SHUYING WU, RULIN YANG, LIPING ZHAO, MINGXING SUI, MANHUA CUI, WEIQIN CHANG
      Oncology Letters.2016; 11(4): 2865.     CrossRef
    • COTYLEDONOID DISSECTING LEIOMYOMA (CDL) OF UTERUS MIMICKING MALIGNANCY: A CLINICAL DILEMMA
      Roma Isaacs, Rupinder Kaur, Sunita Goyal
      Journal of Evolution of Medical and Dental Sciences.2016; 5(57): 3973.     CrossRef

    • PubReader PubReader
    • ePub LinkePub Link
    • Cite this Article
      Cite this Article
      export Copy Download
      Close
      Download Citation
      Download a citation file in RIS format that can be imported by all major citation management software, including EndNote, ProCite, RefWorks, and Reference Manager.

      Format:
      • RIS — For EndNote, ProCite, RefWorks, and most other reference management software
      • BibTeX — For JabRef, BibDesk, and other BibTeX-specific software
      Include:
      • Citation for the content below
      Cotyledonoid Dissecting Leiomyoma of the Uterus with Intravascular Luminal Growth: A Case Study
      Korean J Pathol. 2013;47(5):477-480.   Published online October 25, 2013
      Close
    • XML DownloadXML Download
    Figure

    J Pathol Transl Med : Journal of Pathology and Translational Medicine