An unusual case of uterine cotyledonoid dissecting leiomyoma with adenomyosis
- Ai Shimizu†1,
- Hoshihito Tanaka†2,
- Sari Iwasaki1Email authorView ORCID ID profile,
- Yukio Wakui2,
- Hitoshi Ikeda1, 3 and
- Akira Suzuki1
© The Author(s). 2016
Received: 19 April 2016
Accepted: 31 July 2016
Published: 4 August 2016
Cotyledonoid dissecting leiomyoma is a rare variant of uterine smooth muscle tumor with an unusual growth pattern that shows intramural dissection within uterine myometrium and often a placenta-like appearance in its extrauterine components.
We present a unique case of cotyledonoid dissecting leiomyoma with adenomyosis. A 40-year-old Japanese female presented with prolonged menorrhagia and severe anemia. She had a pelvic mass followed-up for 6 years with a diagnosis of leiomyoma. However, increase in tumor size and cystic changes with hemorrhage were found by magnetic resonance imaging, and total abdominal hysterectomy with bilateral salpingectomy was performed. Macroscopically, the placenta-like exophytic mass protruding from the posterior uterine wall was composed of multiple nodules containing numerous hemorrhagic cysts. The mass showed continuity as a white multinodular dissecting mass infiltrating the posterolateral myometrium. Microscopically, both extra–and intrauterine portions of the mass were composed of nodules that contained swirled neoplastic smooth muscle cells with marked hyalinized degeneration, as observed in cotyledonoid dissecting leiomyomas of conventional type. In addition, numerous non–neoplastic glands of endometrial type surrounded by abundant endometrium–like stromal cells and non–neoplastic smooth muscle cells were found in the tumor, suggesting that it involved a part of concomitant adenomyosis originating from the nontumoral myometrium.
Thus far, over 30 cases of cotyledonoid dissecting leiomyoma have been reported, none of which have described the presence of adenomyosis within the tumor. The present case suggested that cotyledonoid dissecting leiomyoma might have a unique clinical presentation involving concomitant uterine adenomyosis. It is critical for pathologists, gynecologists, and radiologists to be cognizant of cotyledonoid dissecting leiomyoma variants for timely and appropriate diagnosis and treatment.
Uterine leiomyomas are the most common type of uterine tumor , with its variants accounting for approximately 10 % of cases . Cotyledonoid dissecting leiomyoma is an extremely rare variant of uterine leiomyoma with an unusual pattern that is characterized by intramural dissection within the uterine corpus and often a placenta–like macroscopic appearance of its extrauterine component . David et al. originally reported this variant as grape–like leiomyoma in 1975 , which was later named as cotyledonoid dissecting leiomyoma in a series of four cases by Roth et al. . Thereafter, over 30 cases have been reported thus far; all followed a clinically benign course [4–6]. Although several cases indicated coexisting focal endometriosis, endosalpingiosis, and adenomyomatous components [7–9], none showed obvious and abundant nontumoral components within the tumor. Here we present a unique case of cotyledonoid dissecting leiomyoma involving numerous non-neoplastic endometrial glands with endometrial stromal cells, indicating adenomyosis. To the best of our knowledge, this is the first description of cotyledonoid dissecting leiomyoma with ademomyosis.
In the present case, the clinical manifestation, gross appearance, and histological findings were in good agreement with typical cotyledonoid dissecting leiomyoma. Leiomyomas of the uterus are the most common uterine tumor and usually affect women in their fourth and fifth decades . Most uterine leiomyomas are conventional types, whereas variant forms comprise approximately 10 % of all leiomyoma cases . Cotyledonoid dissecting leiomyoma is an extremely rare variant of uterine leiomyoma with an unusual growth pattern characterized by an extrauterine mass that resembles a placenta and histologically shows an intramural dissecting pattern in the uterus [2, 3]. Saeed et al. reviewed 20 cases of cotyledonoid dissecting leiomyoma . The ages of patients ranged from 23 to 65 years (mean, 40.3 years), which were less than those of patients with conventional leiomyomas. The most common clinical presentation of this variant is a pelvic mass and is followed by abnormal uterine bleeding. The size of these tumors ranged from 10 to 41 cm (mean, 17.7 cm). Thus far, over 30 cases have been reported in the literature [4, 5]. Histologically, the neoplastic smooth muscle cells form disorganized fascicles in cotyledonoid dissecting leiomyomas, in contrast to the organized pattern observed in conventional leiomyomas . In this case, both the intrauterine and extrauterine part of the tumor was composed of the same nodular masses of disorganized smooth muscle cells and collagen fibers as those of previous reports (Fig. 2c and d). All cases reported to date were clinically benign, and only one case of recurrence after partial tumorectomy was reported . Cases of epithelioid cotyledonoid dissecting leiomyoma variant  and cotyledonoid dissecting leiomyoma with intravenous leiomyomatosis  were also recently reported. However, neither an epithelioid pattern nor intravascular tumor components were found in our case.
Summary of all reported cases of cotyledonoid dissecting leiomyoma with non-neoplastic cystic lesions
Abdominal pain and mass
Endometriosis in left ovary
Posterior wall, broad ligaments, pelvic cavity
Menorrhagia and severe anemia
Posterolateral wall, pelvic cavity
We suppose the endometrial component in this tumor may be a part of adenomyosis, not endometriosis. It is because localization of the endometrial component is not superficial and endometriosis is not found in any part of the pelvic cavity. However, adenomyosis is distributed both in the tumor and tumor-free myometrium (Fig. 3d). Furthermore, some of these endometrial elements within the tumor are enclosed by non-neoplastic myometrial smooth muscle that is slightly immunopositive for Bcl-2 (Fig. 4a and b). Bcl-2 protein is known to be an apoptosis-inhibiting gene product, and it is also known to prevent apoptotic cell death in a variety of cells. Matsuo et al. reported that Bcl-2 immunopositivity was prominent in uterine leiomyoma cells and was scarcely present in normal myometrial smooth muscle cells . They supposed that Bcl-2 protein associated with progesterone is responsible for the growth of leiomyomas by preventing apoptotic cell death .
On the other hand, adenomyoma should be an important differential diagnosis in the extrauterine part of the tumor. Adenomyoma is one of the mixed epithelial and mesenchymal tumors of the uterine corpus and is described as a well-circumscribed tumor composed of endometrial glands and endometrial-type stroma surrounded by abundant smooth muscle component . We cannot definitely distinguish adenomyoma with involved adenomyosis in a cotyledonoid dissecting leiomyoma, because each histological component is very similar and also because of degenerative changes in the extrauterine part caused by menstrual bleeding followed by inflammation. Typical adenomyomas are intramural, firm, and smooth-surfaced tumors . They show gray-white surfaces and are well demarcated from the myometrium on cut sections, and their smooth muscle component shows hypertrophy but does not make nodular proliferation with collagen fibers . However, we can also find a few previous case reports of adenomyoma with prominent cystic change extended to the pelvic cavity [16, 17]. Since histological analysis of these tumors is not provided in detail, we cannot distinguish them from cotyledonoid dissecting leiomyoma with adenomyosis. Accumulation of similar cases will help us understand the nature and the difference between adenomyoma and cotyledonoid dissecting leiomyoma with adenomyosis.
The mechanism of tumor development in this patient is interesting. In this case, leiomyoma was first found in the uterine wall by transvaginal ultrasonography and was later noted to extend into the pelvic cavity. It is possible that during tumor growth from the myometrium, endometrial glands and stromal cells might have been captured by the intramural component of tumor during its dissection through the surrounding adenomyosis, to be located together with the extrauterine component. In this case, glandular components and stromal cells were also recognized in the intramural component, supporting this possibility. The cystic expansion of endometrial glands outside the uterus might be correlated with loose connective tissue with congestion and hydropic change.
The cotyledonoid dissecting leiomyoma in our case included a large number of non-neoplastic cystic lesions compared with previously reported three cases. This case indicated that cotyledonoid dissecting leiomyoma could occur with concomitant adenomyosis, depending on the pattern of tumor growth. These four cases, including ours, suggested that cotyledonoid dissecting leiomyoma might have a unique presentation with benign non-neoplastic cystic lesions, such as endometriosis, endosalpingiosis, adenoleiomyomatous component, and adenomyosis.
The case presented herein is the first documented cotyledonoid dissecting leiomyoma with diffuse adenomyosis and reveals a unique variant of cotyledonoid dissecting leiomyoma involving benign cystic lesions. Accumulation of similar case reports is expected and important. While cotyledonoid dissecting leiomyoma has alarming gross appearance with hemorrhagic cysts of adenomyosis or other benign cystic lesions within the tumor mass, it has a completely benign clinical course. Gynecologists, radiologists, and pathologists should be cognizant of the variance and nature of cotyledonoid dissecting leiomyomas to avoid misdiagnosis and overtreatment.
Bcl-2, B-cell lymphoma 2; CD10, Cluster of differentiation 10
We would like to thank Drs. Shinichi Tejima, Akio Sakatani, Megumu Fujiwara, Yasunari Oda, and Kanako Hatanaka for their valuable advice. We also thank Mr. Makoto Imagawa, Mr. Atushi Minoshima, Ms. Madoka Yamaguchi, Ms. Yukiko Oda, Mr. Jun Koizumi, Mr. Yuji Meguro, and Ms. Kuniko Asahi for providing sections for routine hematoxylin-eosin staining and immunohistochemistry at the Department of Pathology of KKR Sapporo Medical Center. We thank Enago for English language review.
This work received no funding support from external sources.
Availability of data and materials
Data and materials of this work are available from the corresponding author on reasonable request.
AS and HT designed and drafted the manuscript. SI, HI, and AS performed histopathological diagnosis and helped in preparing the manuscript. YW collected clinical data. All authors read and approved the final manuscript.
The authors declare that they have no competing interests.
Consent for publication
Written informed consent was obtained from the patient for publication of this Case Report and any accompanying images.
Ethics approval and consent to participate
A statement of ethics approval for this case report by the Ethics committee of KKR Sapporo Medical Center is available on request (reference number 27–36).
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