Cotyledonoid Dissecting Leiomyoma of the Uterus

Abstract

Cotyledonoid dissecting leiomyoma is a rare benign uterine tumor whose gross and radiological appearance may raise the possibility of a malignancy. The authors summarize herein the clinical, radiological, and pathological features of the 41 previously reported cases. The patients typically presented with menorrhagia or symptoms that were ultimately relatable to the presence of a pelvic mass. The median patient age was 46 years (range 23-73). The average tumor size was 15.4 cm (range 4-41); most were exophytic, multinodular, occasionally cystic masses with a congested, spleen or placenta-like color, protruding over the uterine serosa and, variably, the broad ligaments and adjacent organs. In most cases, the exophytic component was contiguous with the intramural dissecting leiomyomatous components. Histological features suggestive of malignancy, such as cytological atypia, necrosis, or increased mitotic activity were absent. Intravascular growth, as assessed histologically, was present in 20% of reported cases, and this finding is apparently devoid of clinical significance based on limited data. Reported information on radiological features is limited. However, these lesions are typically isointense to myometrium on T1-weighted MRI and are in general less heterogeneous on T2 and postcontrast-imaging than sarcomatous lesions. The tumor may display extension to but not frank invasion of surrounding organs. Ultrasound features are nonspecific. Follow-up information was available in 25 (61%) of 41 reported patients: none experienced a tumor recurrence or metastases during the follow-up period, which ranged from 1 month to 41 years. This affirms the benign nature of this tumor, its alarming gross appearance and growth patterns notwithstanding.

Keywords

cotyledonoid dissecting hydropic leiomyoma perinodular uterus

Introduction

Leiomyomas of the uterus may display a variety of growth patterns other than the typical appearance of a well-circumscribed, uniformly expansile tumor.¹ Leiomyomas with such unusual growth patterns include diffuse leiomyomatosis of the uterus, leiomyoma with vascular invasion, intravenous leiomyomatosis (IVL), benign metastasizing leiomyoma, parasitic leiomyomas, disseminated peritoneal leiomyomatosis, dissecting leiomyomas, and leiomyomas with a nodular growth pattern and perinodular hydropic degeneration.² Leiomyomas with a dissecting growth pattern were defined in 1 study as smooth-muscle tumors that microscopically display tongues of tumoral tissue extending significantly (at least 5 mm) between fascicles of the myometrium adjacent to the dominant mass.³ These tumors are probably synonymous with the infiltrating leiomyomas as described by Hendrickson and Kempson almost 20 years previously.¹ Dissecting leiomyomas of the uterus may display extrauterine extension or be confined to the uterus.^1-4

Cotyledonoid dissecting leiomyoma (CDL) is a distinctive variant of dissecting leiomyoma that is characterized by a reddish, exophytic, placenta-like gross appearance and by its frequent extrauterine extension into the adjacent tissues and the pelvic cavity.^5-33 CDL was originally described as “grapelike leiomyoma” by David et al⁵ in 1975 and as “proliferating pelvic angioleiomyomatosis (red seaweed lesion)” by Sternberg⁶ in 1979. Sternberg tumor and CDL (the latter based on its placental-like appearance) are both appellations that were initially applied by Roth et al⁷ in their 1996 report of 4 cases that formally defined the lesion as a distinct clinicopathological entity. In total, approximately 43 cases of CDL have been reported, including 41 in the English language literature (Table 1).

Table 1.

Summary of Reported Cases of Cotyledonoid-Dissecting Leiomyoma in the English-Language Literature^a

Reference	Patient Age	Clinical Presentation	Tumor Size (cm)	Tumor Location and Extension	Management	Outcome
David et al⁵	65	Abnormal uterine bleeding and abdominal tenderness	15	Uterine fundus and cervix, bladder peritoneum	TH	NA
	48	Uterine prolapse	12	Uterine fundus, broad ligament, retroperitoneum	TH and tumor resection	NA
Brand et al⁹	24	Abdominal mass and lower-abdominal swelling	NA	Uterine fundus, extending into broad ligaments and peritoneal cavity	Tumor resection (incomplete) with postoperative progestins	No increase in size of residual tumor, 15 months
Roth et al⁷	39	Pelvic mass	10, 3	Uterine surface in the region of the cornu, extending into the broad ligament and projecting into the pelvic cavity	TAH/BSO	ANED 26 months
	41	Abnormal uterine bleeding, enlarged uterus	10	Uterine surface in the region of the cornu, extending into the broad ligament and projecting into the pelvic cavity	TVH	DNED 41 years
	23	Pelvic mass	25	Uterine surface in the region of the cornu, extending into the broad ligament and projecting into the pelvic cavity	Resection	ANED 16 years
	Unknown	Pelvic mass	24	Uterine surface in the region of the cornu, extending into the broad ligament and projecting into the pelvic cavity	TAH/BSO	NA
Menolascino-Bratta et al¹⁰	26	Incidentally detected	16	Uterus, broad ligaments, fallopian tubes, peritoneal surfaces	TAH/BSO	NA
Roth and Reed¹¹	46	Abnormal uterine bleeding, pelvic mass	34, 5.5	Uterus in region of cornu, broad ligament, peritoneal cavity	TAH/BSO; omental biopsy	ANED, 1 month
Cheuk et al¹³	55	Abnormal uterine bleeding, uterine prolapse	10	Uterine surface in the region of the cornu, extending into the broad ligament and projecting into the pelvic cavity	TH/BSO	ANED, 14 months
Kim et al¹²	26	Incidentally detected	12	Posterior uterine wall, left broad ligament and pelvic cavity	Tumor resection	ANED, 2 years
Jordan et al¹⁴	46	Right adnexal mass	22	“Masses within the myometrium extending through the serosal surface in an endoexophytic manner, occasionally extending into the broad ligament or appearing to give rise to a separate adnexal mass” (p. 247)	TAH/BSO	NA
	46	Pelvic mass, weight gain, central abdominal pain	20		TAH/BSO	ANED, 5 years
	46	Pelvic mass	10		TAH and resection of right broad ligament mass	NA
	46	Pelvic mass	18		TAH	NA
	36	Abnormal uterine bleeding, uterine mass	13		Subtotal hysterectomy	NA
	34	Uterine mass, infertility	18		Local resection, followed by TAH	ANED
Stewart et al¹⁵)	58	Abdominopelvic mass, rectal bleeding, stress incontinence	16.4	Uterine fundus	TAH/BSO	NA
Saeed et al¹⁶	27	Pelvic mass	41	Posterior uterine wall, broad ligament, and pelvic cavity	TAH/BSO	ANED, 1 month
Gurbuz et al¹⁷	67	Incidentally detected	10	Protruding into abdominal cavity near the uterine cornu; broad ligament	TH/BSO	ANED, 8 months
Maimoon et al¹⁸	40	Urinary retention, suprapubic fullness	10	Left isthmic region of uterus, cervix, broad ligament	TAH/SO	ANED, 14 months
Weissferdt et al¹⁹	52	Abnormal uterine bleeding, abdominal pain and tiredness	6, 2	Broad ligament, uterine fundus	TAH/LSO	ANED, 18 months
Mathew et al²⁰	Early 30s	Abdominal distension and discomfort	30	Broad ligament, cervix, adherent to surface of left lateral uterine wall, small and large bowel	Tumor resection	ANED, 5 months
Shelekhova et al²¹	73	Uterine mass	8	Uterine fundus, broad ligament, pelvic cavity	NA	NA
	48	Abdominal pain	9	Uterine fundus and cornu, broad ligament	TAH/BSO	ANED, 2 years
Driss et al²²	47	Pelvic mass, abnormal uterine bleeding	25	Uterine mass protruding into pelvic cavity and broad ligaments	TAH/BSO	ANED, 10 months
Raga et al²³	33	Abdominal pain, abnormal uterine bleeding	6	Lateral aspect of uterus, broad ligament	Tumor resection	NA
Preda et al²⁴	41	Uterine masses	9	Left and posterior uterine wall, left broad ligament, abutting rectum posteriorly	TH/Left oophorectomy	NA
Soleymani Majd et al²⁵	63	Abdominal pain, abnormal uterine bleeding	4	Right broad ligament, lateral surface of uterus	TAH/BSO	ANED 6 months
Adedipe and Vine²⁶	37	Abnormal uterine bleeding	NA	Uterine surfaces, left broad ligament	TAH/partial omentectomy	NA
Fukunaga et al²⁷	56	Constipation	30	Exophytic mass, posterior uterus	TH/SO	ANED, 6 months
	47	Abdominal mass	26	Exophytic mass, posterior uterus	TH/SO	ANED, 4 months
	36	Abnormal uterine bleeding	4	Exophytic mass, posterior uterus	TH	ANED, 8 years
	35	Abdominal pain, palpable abdominal mass	18	Exophytic mass, lateral uterus	TH/SO	ANED, 12 years
Agarwal et al²⁸	52	Abnormal uterine bleeding	10	Cornual region of uterus	TVH	NA
Gezginç et al³⁰	57	Pelvic pain	2.5, 4.5	Intrauterine mass; exophytic mass, lateral uterus	TH/BSO	ANED, 22 months
Ersöz et al³¹	51	Abnormal uterine bleeding	8.5	Exophytic mass, uterine serosa	TH/BSO	ANED, 6 months
Aggarwal and Arora³²	52	Abnormal uterine bleeding; uterine prolapse	10	Exophytic mass, uterine serosa, extending into peritoneal cavity and broad ligament	TH/LSO	ANED, 6 months
Nasfi et al³³	50	Abdominal mass	10	Exophytic mass, extending from the uterine surface and projecting into the broad ligaments and the pelvic cavity	Tumor resection	NA
	52	Abnormal uterine bleeding	7	Exophytic mass, extending from the uterine surface and projecting into the broad ligaments and the pelvic cavity	TH	NA
	49	Abdominal mass	30	Exophytic mass, uterine serosa	Subtotal hysterectomy	ANED, 10 months

Abbreviations: TH, total hysterectomy; TAH, total abdominal hysterectomy; BSO, bilateral salpingo-oophorectomy; TVH, total vaginal hysterectomy; ANED, alive with no evidence of disease; DNED, dead with no evidence of disease; SO, salpingo-oophorectomy; LSO, left salpingo-oophorectomy; NA, information not available.

Excludes the case reported by Gowri et al³⁴ because of insufficient clinicopathological information on the tumor.

Clinical Presentation

Of the CDL patients outlined in Table 1, 40 ranged in age from 23 to 73 years (mean 45, median 46 years). The patients typically presented with abnormal uterine bleeding (most frequently menorrhagia) and/or symptoms that were ultimately relatable to the presence of a pelvic mass (including constipation, abdominal pain, weight gain, and palpable abdominopelvic masses^5-28,30-33). Rare presentations included uterine prolapse, infertility, stress urinary incontinence, urinary retention, and rectal bleeding. In 3 patients, the tumors were incidental discoveries during the diagnostic workup for, or management of, other medical issues.^10,12,17

Radiological Features

The ultrasonographic attributes of CDL are not notably distinct from those of conventional leiomyomas, and it is characterized by one or more bulky or lobulated masses with irregular borders.^12,19,23 Preda et al²⁴ detailed the radiographic features of a case of CDL as evaluated by MRI. On T1-weighted sequences, the lesion was homogeneously isointense (relative to the surrounding myometrium) and showed marked contrast enhancement in the images obtained postcontrast. T2-weighted sequences were more heterogeneous, which the authors attributed to hyperintense stromal components and confluent tumor nodules that were isointense to the surrounding myometrium.²⁴ Our general impression of the reported cases is that preoperative imaging was not suggestive of the eventual diagnosis and very often raised the index of suspicion for a malignant process. In 1 recently encountered case at Vanderbilt University Medical Center, a large mass was demonstrated to be arising from the uterus, extending predominantly anteriorly and laterally with some extension to the superior margin of the bladder and adjacent bowel above the uterus. This lesion showed features differing from conventional leiomyomas in the adjacent uterus. Morphological differences included the lack of well-defined margins and the presence of a bizarre lobulated appearance. Signal characteristics also provided a contrast, though less striking, to adjacent well-circumscribed leiomyomas with isointensity on T1 relative to the myometrium. T2 signal characteristics were more variable, with modest heterogeneity, increased signal as compared with adjacent leiomyomas and decreased signal with respect to the myometrium. Following contrast administration, mass enhancement was robust, paralleling the adjacent focal leiomyomas with minimal heterogeneity and near isointense signal relative to the myometrium. The tumor showed extension to but not frank invasion of surrounding organs; a fat plane, albeit small, was seen between the mass and adjacent structures such as the urinary bladder and bowel. There was no evidence of intravascular extension despite the aggressive appearance of this lesion. These features may allow the radiologist to suggest CDL or another less aggressive process in the differential, although biopsy or resection would certainly be required for a definitive diagnosis. (Figures 1A-1C)

Gross Pathology

A typical case of CDL is pictured in Figure 2. In this particular case, the uterus displayed multiple bulky, exophytic, bulbous, red-brown masses protruding from the serosal surface in the left uterine cornu and involving the broad ligament. The largest mass measured 11 × 6 × 4.5 cm³. The myometrium harbored numerous white-tan, whirled, nodular masses. Subserosal conventional leiomyomata were also present. The exophytic masses protruding from the serosa of the uterus were in continuity with intramural masses.

Across a variety of reports, the described macroscopic appearance of CDL has been notably similar to this basic profile. CDL is characterized by exophytic, multinodular, occasionally cystic masses with a congested, spleen or placenta-like color, involving the uterine surfaces and, variably, the broad ligaments, the adnexal organs, the larger abdominopelvic cavity, the retroperitoneum, and the surfaces of the uterine cervix and bladder. The bulbous protrusions that constitute the mass were described as being “loosely attached to their neighbors by thin adhesions and delicate vessels” by Roth et al,⁷ and Fukunaga et al²⁷ noted that small veins are characteristically present in the interlobular regions. The nidus of the mass is frequently in the uterine cornual region, and bilaterality is common. This exophytic mass is often contiguous with one or more uterine intramural leiomyomatous masses,^7,27 but the focus of continuity may not be immediately apparent, resulting in the potential appearance of a separate adnexal mass.¹⁴ Additionally, rare examples of CDL that presumably originated from subserosal smooth muscle may lack a parent leiomyomatous mass or an intramural dissecting component.^11,17,18,33 The reported tumors have ranged in size from 4 to 41 cm (average 15.4, median 12 cm; Table 1). Conventional leiomyomas, which may be numerous and widely distributed, are frequently present. The gross appearance of the intramural component is variable and is largely a function of the type of leiomyoma that is so classified. A conventional intramural dissecting leiomyoma may have a solid appearance with a whorled, tan, cut surface that is distinguishable from a conventional leiomyoma only by its infiltrative edges. However, they may also be notably edematous, variably cystic, and/or only focally infiltrative.

Histopathology

The typical microscopic appearance of the cotyledonoid component of CDL is of poorly cohesive round nodules of benign smooth muscle that are surrounded by loose connective tissue with congested vessels (Figure 3A). When tissue sections are able to capture a nonfragmented portion of the cotyledonoid component, nodules of variably cellular smooth muscle can be seen separated by hydropic fibrous tissue, an appearance that is reminiscent of perinodular hydropic degeneration. (Figure 3B). Numerous vessels of varying caliber may be seen in the central portions of the nodules,⁷ and florid edema may significantly separate the vessels.¹⁴ Hydropic change of varying extents were stated to be present in 29 cases, and no information on hydropic change was given in the remaining 12. Constituent cells are most frequently spindled smooth-muscle cells lacking cytological atypia (Figure 3C), but a single case of an epithelioid CDL has been described.²⁵ The spindle cells show a higher level of disorganization than is typical for a conventional leiomyoma, and there is often stromal hyalinization (Figure 3D).^7,14,27 Neurilemmoma-like cellular margination or “swirling” is not uncommon, and adipocytic differentiation was present in 1 case.²⁷ None of the reported cases displayed an increased mitotic index (>5 mitotic figures/10 high-power microscopic fields) or tumor cell necrosis. Approximately 90% of the reported cases of CDL had an intramural dissecting component. This component comprises infiltrating, sinus-like, haphazard tongues of smooth muscle in the background myometrium (Figure 3E). As noted by Roth et al,⁷ in a given tissue section, the tongues appeared as “rounded, ovoid, or elongated processes.” These changes may be focal, at the edge of a conventional leiomyoma, or may comprise the entire intramural mass. Some authors have required that the dissecting component extends for at least 5 mm.⁷ Three cases were associated with endosalpingiosis^22,27,33 and 2 with endometriosis,^27,33 but it is unclear if these associations were entirely fortuitous. One uterine tumor, interpreted as a CDL with an “adenoleiomyomatous” element, extended into the adnexa resulting in a cystic mass lined by a tuboendometrial epithelium.¹⁴ Intravascular growth, typically focal (Figure 3F), was present in 8 (20%) of the reported 41 cases.^14,21,27,30

Pathological Differential Diagnosis

The most significant differential diagnostic considerations for CDL, especially if encountered in a biopsy, include hydropic leiomyomas and leiomyoma with perinodular hydropic degeneration (LPHD), myxoid lesions, IVL, low-grade endometrial stromal sarcoma (LGESS), perivascular epithelioid cell tumors (PEComas), and leiomyosarcomas.

Hydropic Leiomyomas, LPHD, and IVL

Hydropic leiomyomas are characterized by a prominent accumulation of intercellular fluid that may be so extensive as to cause cystic degeneration.³⁵ As previously noted, CDLs commonly display hydropic change. Pure hydropic leiomyomas, however, do not display intramural dissecting or extramural cotyledonoid components. The typical gross appearance of a hydropic leiomyoma is a circumscribed mass with a tan, solid, occasionally cystic cut surface.³⁵ LPHDs are characterized by nodules of variably hydropic smooth muscle surrounded by markedly hydropic stroma.^35-37 When LPHDs are extrauterine, they may have a gross and microscopic appearance that renders them essentially indistinguishable from a CDL. As discussed below, we believe that CDL and LPHD are related lesions. IVL is defined by an intravascular growth pattern of benign smooth muscle outside of the confines of a main leiomyomatous mass.^38-40 The extensive perinodular hydropic change in LPHD can result in some overt degeneration around individual nodules, and as such, they may appear to be in vascular spaces, simulating IVL. As previously noted, approximately 20% of CDLs display intravascular growth; those lesions where the intravascular growth is outside the main tumoral mass should be said to display an IVL component. Those lesions in which the intravascular growth is within the body of the tumor should be stated as displaying vascular invasion. It is probable that CDL, LPHD, and IVL are pathogenetically related.⁷ All 3 tumors may display a mural dissecting growth pattern, extrauterine extension, a cotyledonoid appearance, lymphovascular invasion, and tumoral hydropic change (Table 2). Indeed, in the largest study of uterine dissecting, noncotyledonoid leiomyomas reported to date,³ of the 8 cases so classified, 4 were intramural dissecting leiomyomas, 3 were examples of IVL, and 1 was a multinodular leiomyoma with hydropic degeneration. This overlap has also resulted in the description of lesions that are “intermediate” or that combine characteristics of any 2 or all of these lesions. Nevertheless, apart from the aforementioned exceptions, both LPHD and CDL should be distinguished from IVL because the latter has a well-defined clinicopathological profile, including rare, isolated metastases and extension through the vena cava and into the heart, with cardiac manifestations that may be seen many years after extirpation of the original uterine tumor.^38,41 Additionally, IVL has been associated with recurrence, and although the recurrence rate is low,⁴⁰ it has nevertheless not been documented in the other 2 tumors.

Table 2.

A Comparison of the Clinicopathological Features That Have Been Described for Intravenous Leiomyomatosis, Leiomyoma With Perinodular Hydropic Degeneration, and Cotyledonoid-Dissecting Leiomyoma

Lesion	Intratumoral Hydropic Change	Hydropic Change in Myometrium	Extrauterine Extension Into Pelvic Tissues	Extrauterine Extension Above the Diaphragm	Lymphovascular Involvement	Nodular Growth Pattern	Placenta-Like Gross Appearance	Mural Dissecting Growth Pattern
Intravenous leiomyomatosis^38-42	Present^a	Absent	Present	Present	Present	Present	Present	Present
Leiomyoma with perinodular hydropic degeneration^35-37	Present	Present	Present	Absent	Present	Present	Present	Present
Cotyledonoid-dissecting leiomyoma^5-33	Present	Absent	Present	Absent	Present	Present	Present	Present

“Present” denotes a feature that has previously been described in the lesion, irrespective of its frequency.

Low-Grade Endometrial Stromal Sarcoma

The dissecting pattern of growth in CDLs within the myometrium as well as their frequently high vascularity are areas of morphological overlap with LGESSs. CDLs may also display significant hydropic change,⁴ and accordingly, mimic myxoid LGESS.⁴³ The constituent cells of conventional LGESS are oval to fusiform and have a diffuse growth pattern, whereas the cells of CDLs are predominantly spindled and are configured in variably disorganized fascicles. Numerous thin-walled arterioles are typical of stromal neoplasms. The vessels of CDLs may be as numerous, but they are typically of a thicker caliber and are less uniformly distributed. In the absence of overt smooth-muscle differentiation, most LGESS have a CD10-positive/desmin-negative/h-caldesmon-negative immunophenotype, whereas most smooth-muscle neoplasms display the converse.

Perivascular Epithelioid Cell Tumors

Some PEComas may display an infiltrative growth pattern within the uterus that is reminiscent of dissecting leiomyoma.^44,45 PEComas comprise admixures of epithelioid and spindle cells, whereas CDLs are mostly constituted of spindle cells, although 1 case of an epithelioid CDL was reported by Soleymani Majd et al. 25 PEComas are defined in part by a mixed myogenic/melanocytic immunophenotype and are positive for desmin in 49% of cases and for melanocytic markers (such as HMB-45 and Melan-A) in 100% and 24% of tested cases, respectively .45 Conventional leiomyomas are of course typically desmin positive and are known to display some expression of melanocytic markers, typically in a weak and/or focal pattern for the latter.^46-48 Features favoring PEComa include a plexiform vascular pattern, presence of the tuberous sclerosis complex in the patient, degenerative multinucleated or “spider” cells, and diffuse expression of melan-A.⁴⁵ Additionally, tumor necrosis, cytological atypia, and a high mitotic index—features that characterize a potentially aggressive subset of PEComas—have not been described in CDLs.

Leiomyosarcoma and Other Lesions

Although the gross appearance of CDLs may raise the definite possibility of malignancy during their intraoperative evaluation, their distinctive microscopic appearance allows them to be clearly defined as benign, in that CDLs do not display morphological features diagnostic of conventional spindle-cell leiomyosarcoma (diffuse cytological atypia, a high mitotic index, and tumor cell necrosis).¹ Myxoid leiomyosarcomas, however, may not display any of these features in a biopsy and may only be distinguishable from hydropic leiomyomas by the recognition of the myxoid, nonedematous nature of the intercellular material and/or lymphovascular invasion.⁴⁹ Other myxoid lesions that are also worthy of consideration in a biopsy include the rare uterine myxomas,^50,51 myxoid leiomyomas,⁵² and inflammatory myofibroblastic tumor of the uterus.⁵³ Myxomas are exceptionally rare at this location and are characterized by a hypocellular proliferation of spindle to stellate cells in a myxoid background. Immunohistochemically, they display no smooth-muscle differentiation. Myxoid leiomyomas are also uncommon, may be associated with pregnancy, are grossly circumscribed, and are microscopically characterized by spindle cells separated by myxoid (nonedematous) material. However, the myxoid change may be focal. Leiomyomas in patients treated with progestins may display hydropic and/or myxoid changes adjacent to foci of infarct-type necrosis.⁵⁴ Unlike CDLs, neither myxoid leiomyomas nor myxomas should display an intramural dissecting or extramural cotyledonoid component. Some features of inflammatory myofibroblastic tumors that may cause them to mimic CDLs include an infiltrative growth pattern into the myometrium, a myxoid background, and microscopic patterns (hypocellular, fascicular, or hyalinized). Unlike CDLs, inflammatory myofibroblastic tumors uniformly express the anaplastic lymphoma kinase protein, typically have a lymphoplasmacytic infiltrate⁵³ and do not display extramural cotyledonoid components.

The etiopathogenesis of CDLs remains largely unknown. In their seminal report, Roth et al⁷ speculated that CDLs originate from the corpus, then extend along the muscular vessels of the middle portion of the myometrium into the broad ligament. At extrauterine sites, venous drainage may become hampered, and congestion may develop. In the cotyledonoid portion, the widely dispersed muscular fibers, the fine fibrous matrix, the high vascularity of discrete areas, and the overlying vascular congestion all combine to result in a placenta-like appearance.

Treatment, Prognosis, and Outcome

Follow-up information was available for 25 (61%) of the 41 reported patients. Of these patients, 6 had been initially treated with a tumor resection only or a subtotal hysterectomy. The 19 others were treated with a total hysterectomy and (at minimum) a unilateral salpingo-oophorectomy; 2 others received only a total hysterectomy. None of the patients for whom follow-up information was available experienced a tumor recurrence or metastases during the follow-up period, which ranged from 1 month to 41 years. This favorable outcome is apparently independent of the presence or absence of conventionally adverse pathological prognostic factors, such as broad extrauterine extension. CDL with lymphovascular invasion did not appear to be prognostically distinct from the other cases in the group, but there were only 8 cases with this finding.^14,21,27,30 Follow-up information was available in 5 of the 6 patients who were treated with tumor resection only, and none had experienced a recurrence or metastases at 10 months (30), 15 months (7), 16 years (9), 2 years (12), and 5 months (20) after their procedures. These findings affirm that CDL is a benign tumor that has minimal, if any, recurrence or metastatic potential after its complete resection.

Summary

CDLs are rare, benign uterine tumors whose gross or radiological appearance may simulate a malignant process. They are most frequently associated with a mural dissecting leiomyomatous component in the uterus and, at the microscopic level, are readily separable from lesions in the differential diagnosis. Familiarity with this distinctive tumor is important: the macroscopic attributes of this tumor are sufficiently distinctive as to allow the gross pathologist to at least suggest the diagnosis before microscopic examination (eg, during intraoperative evaluation). Similarly, some radiological features may allow the radiologist to suggest CDL or another nonaggressive process in the differential prior to a biopsy or definitive resection. A review of the outcomes for previously reported cases confirmed that CDL is a benign tumor.

Footnotes

Declaration of Conflicting Interests

The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Funding

The author(s) received no financial support for the research, authorship, and/or publication of this article.

References

Hendrickson

Kempson

. Smooth muscle neoplasms. In: Hendrickson

Kempson

. Surgical Pathology of the Uterine Corpus. Philadelphia, PA: W B Saunders; 1980:468-529.

Tse

Tam

. Uterine smooth muscle tumors other than the ordinary leiomyomas and leiomyosarcomas: a review of selected variants with emphasis on recent advances and unusual morphology that may cause concern for malignancy. Adv Anat Pathol. 2010;17:91-112.

Roth

Reed

. Dissecting leiomyomas of the uterus other than cotyledonoid dissecting leiomyomas: a report of eight cases. Am J Surg Pathol. 1999;23:1032-1039.

Fukunaga

Ushigome

. Dissecting leiomyoma of the uterus with extrauterine extension. Histopathology. 1998;32:160-164.

David

Homonnai

Deligdish

Loewenthal

. Grape-like leiomyomas of the uterus. Int Surg. 1975;60:238-239.

Sternberg

. Proliferating pelvic angioleiomyomatosis (red seaweed lesion). Proceedings of the 9th George Papanicolaou Memorial Seminar in Gynecologic Pathology. Las Vegas, Nevada, November 7, 1979.

Roth

Reed

Sternberg

. Cotyledonoid dissecting leiomyoma of the uterus: the Sternberg tumor. Am J Surg Pathol. 1996;20:1455-1461.

Payan

Monges

Jouve

Sudan

Gamerre

. An unusual case of uterine leiomyoma: exophytic grapelike development in the pelvic peritoneum. Arch Anat Cytol Pathol. 1980;28:45-49.

Brand

Scurry

Planner

Grant

. Grapelike leiomyoma of the uterus. Am J Obstet Gynecol. 1995;173:959-961.

10.

Menolascino-Bratta

García de Barriola

Naranjo de Gómez

García Tamayo

Suarez

Hernández Chacón

. Cotyledonoid dissecting leiomyoma (Sternberg tumor): an unusual form of leiomyoma. Pathol Res Pract. 1999;195:435-438.

11.

Roth

Reed

. Cotyledonoid leiomyoma of the uterus: report of a case. Int J Gynecol Pathol. 2000;19:272-275.

12.

Kim

Park

Cho

. Cotyledonoid dissecting leiomyoma of the uterus: a case report and review of the literature. J Korean Med Sci. 2002;17:840-844.

13.

Cheuk

Chan

Liu

. Cotyledonoid leiomyoma: a benign uterine tumor with alarming gross appearance. Arch Pathol Lab Med. 2002;126:210-213.

14.

Jordan

Al-Nafussi

Beattie

. Cotyledonoid hydropic intravenous leiomyomatosis: a new variant leiomyoma. Histopathology. 2002;40:245-252.

15.

Stewart

Ireland-Jenkin

Quinn

Armes

. Cotyledonoid dissecting leiomyoma. Pathology. 2003;35:177-179.

16.

Saeed

Hanaa

Faisal

Najla

. Cotyledonoid dissecting leiomyoma of the uterus: a case report of a benign uterine tumor with sarcoma like gross appearance and review of literature. Int J Gynecol Pathol. 2006;25:262-267.

17.

Gurbuz

Karateke

Kabaca

Arik

Bilgic

. A case of cotyledonoid leiomyoma and review of the literature. Int J Gynecol Cancer. 2005;15:1218-1221.

18.

Maimoon

Wilkinson

Mahore

Bothale

Patrikar

. Cotyledonoid leiomyoma of the uterus. Indian J Pathol Microbiol. 2006;49:289-291.

19.

Weissferdt

Maheshwari

Downey

Rollason

Ganesan

. Cotyledonoid dissecting leiomyoma of the uterus: a case report. Diagn Pathol. 2007;2:18.

20.

Mathew

Gowri

Al Hamdani

Machado

Rao

Shabnam

. Cotyledonoid leiomyoma in pregnancy. Obstet Gynecol. 2007;109:509-511.

21.

Shelekhova

Kazakov

Michal

. Cotyledonoid dissecting leiomyoma of the uterus with intravascular growth: report of two cases. Virchows Arch. 2007;450:119-121.

22.

Driss

Zhioua

Doghri

Mrad

Dhouib

Romdhane

. Cotyledonoid dissecting leiomyoma of the uterus associated with endosalpingiosis. Arch Gynecol Obstet. 2009;280:1063-1065.

23.

Raga

Sanz-Cortés

Casañ

Burgues

Bonilla-Musoles

. Cotyledonoid dissecting leiomyoma of the uterus. Fertil Steril. 2009;91:1269-1270.

24.

Preda

Rizzo

Gorone

Fasani

Maggioni

Bellomi

. MRI features of cotyledonoid dissecting leiomyoma of the uterus. Tumori. 2009;95:532-534.

25.

Soleymani Majd

Ismail

Desai

Reginald

. Epithelioid cotyledonoid dissecting leiomyoma: a case report and review of the literature. Arch Gynecol Obstet. 2011;283:771-774.

26.

Adedipe

Vine

. Dissecting cotyledonoid leiomyoma: a rare cause of chronic intractable menorrhagia (not amenable to medical treatment): case report. Eur J Gynaecol Oncol. 2010;31:230-232.

27.

Fukunaga

Suzuki

Hiruta

. Cotyledonoid dissecting leiomyoma of the uterus: a report of four cases. APMIS. 2010;118:331-333.

28.

Agarwal

Radhika

Malik

Radhakrishnan

. Cotyledonoid leiomyoma and non-descent vaginal hysterectomy. Arch Gynecol Obstet. 2010;281:971-972.

29.

Cai

Zhang

Weng

. Cotyledonoid hydropic intravenous leiomyomatosis of uterus: report of a case [in Chinese]. Zhonghua Bing Li Xue Za Zhi. 2006;35:763-764.

30.

Gezginç

Yazici

Selimoğlu

Tavli

. Cotyledonoid dissecting leiomyoma of the uterus with intravascular growth in postmenopausal woman: a case presentation. Int J Clin Oncol. 2011;16:701-704.

31.

Ersöz

Turgutalp

Mungan

Güvendı

Güven

. Cotyledonoid leiomyoma of uterus: a case report. Turk Patoloji Derg. 2011;27:257-260.

32.

Aggarwal

Arora

. An unusual variant of leiomyoma masquerading peroperatively as sarcoma. Indian J Cancer. 2011;48:120-121.

33.

Nasfi

Sellami-Dhouib

Doghri

. Cotyledonoid dissecting leiomyoma of the uterus: a report of four cases. Case Rep Pathol. In press.

34.

Gowri

Al Dughaishi

Arumugam

Mathew

. Misdiagnosed cervical pregnancy following a pregnancy with cotyledonoid leiomyoma. Eur J Obstet Gynecol Reprod Biol. 2011;159:241-242.

35.

Clement

Young

Scully

. Diffuse, perinodular, and other patterns of hydropic degeneration within and adjacent to uterine leiomyomas: problems in differential diagnosis. Am J Surg Pathol. 1992;16:26-32.

36.

Ceyhan

Simşir

Dölen

Calýşkan

Umudum

. Multinodular hydropic leiomyoma of the uterus with perinodular hydropic degeneration and extrauterine extension. Pathol Int. 2002;52:540-543.

37.

Coad

Sulaiman

Das

Staley

. Perinodular hydropic degeneration of a uterine leiomyoma: a diagnostic challenge. Hum Pathol. 1997;28:249-251.

38.

Harper

Scully

. Intravenous leiomyomatosis of the uterus: a report of four cases. Obstet Gynecol. 1961;18:519-529.

39.

Norris

Parmley

. Mesenchymal tumors of the uterus: V. Intravenous leiomyomatosis. A clinical and pathologic study of 14 cases. Cancer. 1975;36:2164-2178.

40.

Clement

Young

Scully

. Intravenous leiomyomatosis of the uterus: a clinicopathological analysis of 16 cases with unusual histologic features. Am J Surg Pathol. 1988;12:932-945.

41.

Worley

Jr Aelion

Caputo

. Intravenous leiomyomatosis with intracardiac extension: a single-institution experience. Am J Obstet Gynecol. 2009;201:574.e1-e5.

42.

Shi

Xiu

Fang

Wang

. Intravenous leiomyomatosis with intracardiac extension: a report of two cases. J Card Surg. 2011;26:56-60.

43.

Oliva

Clement

Young

. Endometrial stromal tumors: an update on a group of tumors with a protean phenotype. Adv Anat Pathol. 2000;7:257-281.

44.

Vang

Kempson

. Perivascular epithelioid cell tumor (“PEComa”) of the uterus: a subset of HMB-45-positive epithelioid mesenchymal neoplasms with an uncertain relationship to pure smooth muscle tumors. Am J Surg Pathol. 2002;26:1-13.

45.

Fadare

. Perivascular epithelioid cell tumor (PEComa) of the uterus: an outcome-based clinicopathologic analysis of 41 reported cases. Adv Anat Pathol. 2008;15:63-75.

46.

Smolarek

Bejarano

Heffelfinger

. HMB-45 immunoreactivity is present in both uterine leiomyomas and normal myometrium [abstract]. Mod Pathol. 1999;12:125.

47.

Williams

Gainey

Lamb

. HMB-45 immunoreactivity in uterine mesenchymal tumors [abstract]. Mod Pathol. 2000;13:120.

48.

Oliva

Wang

Branton

. Expression of melanocytic (“PEComa”) markers in smooth muscle tumors of the uterus: an immunohistochemical analysis of 86 cases [abstract]. Mod Pathol. 2006;86:191A.

49.

Burch

Tavassoli

. Myxoid leiomyosarcoma of the uterus. Histopathology. 2011;59:1144-1155.

50.

Rokitansky

. On the occurrence of primary pure myxoma of the uterus. Krebsarzt. 1951;6:341-345.

51.

Barlow

Abu-Gazeleh

Tam

. Myxoid tumor of the uterus and right atrial myxomas. S D J Med. 1983;36:9-13.

52.

Atkins

Bell

Kempson

. Myxoid smooth muscle tumors of the uterus [abstract]. Mod Pathol. 2001;14:132A.

53.

Rabban

Zaloudek

Shekitka

Tavassoli

. Inflammatory myofibroblastic tumor of the uterus: a clinicopathologic study of 6 cases emphasizing distinction from aggressive mesenchymal tumors. Am J Surg Pathol. 2005;29:1348-1355.

54.

Boyd

McCluggage

. Unusual morphological features of uterine leiomyomas treated with progestogens. J Clin Pathol. 2011;64:485-489.