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					1    Title : COTYLEDONOID DISSECTING LEIOMYOMA OF THE UTERUS: A REPORT OF FOUR
2    CASES

3    Article type: Cases report

4    Keywords: dissecting leiomyoma, leiomyoma, endometriosis, endosalpingiosis

5    Corresponding Author : Dr Asma Nasfi

6    Corresponding Author’s Institution: Salah Azaiz Institute

7    First Author : Asma Nasfi

 8   Order of Authors : Asma Nasfi; Rym Sellami-Dhouib, MD; Raoudha Doghri, MD; Samia Sassi, MD;
 9   Lamia Charfi, MD; Imen Abbes, MD; Karima Mrad, Pr ; Naziha Ben Hamida, Khaled Ben
10   Romdhane, Pr
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76    Title:   COTYLEDONOID DISSECTING LEIOMYOMA OF THE UTERUS: A REPORT OF FOUR
77    CASES

78    Authors:     Asma Nasfi; Rym Sellami-Dhouib; Raoudha Doghri, Samia Sassi, Lamia Charfi, Imen
79    Abbes, Karima Mrad, Naziha Ben Hamida, Khaled Ben Romdhane.

80    Institutions and affiliation: Department of pathology, Salah Azaïz Institute,
81    1006 Bab Saadoun,Tunis, Tunisia
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83    Corresponding Author: Asma Nasfi, Resident in Pathology
84    Department of Pathology
85    Institut Salah Azaiz
86    1006 Bab Saadoun, Tunis, Tunisia
87    Tel: 0021698635868
88    Fax: 00 216 71 571 380
89    E- mail: nasfiasma@yahoo.fr

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108   ABSTRACT

109   OBJECTIV E
110   Conventional leiomyomas are the most frequent neoplasm of the female genital tract.
111   Some variant forms of uterine leiomyoma with unusual infiltrative growth pattern have
112   been known, including intravenous leiomyomatosis, diffuse leiomyomatosis,
113   leiomyoma with vascular invasion and disseminated peritoneal leiomyomatosis.
114   Cotyledonoid dissecting leiomyoma (Sternberg tumor) was proposed for a new form
115   of uterine leiomyoma with histologically benign but distinctive and alarming gross
116   features resembling cotyledons of the placenta.
117   We report herein four additional cases of this distinctive benign uterine tumor with
118   unusual growth pattern and some histopathological particularities, occasionally
119   causing diagnostic confusion for pathologists who had not experienced such a tumor
120   before.

121   METHODS
122   We report four new cases of this distinctive benign uterine tumor with unusual growth
123   patterns, reported in Salah Azaiz Institute department of pathology during the period
124   between 2006 and 2011. The clinicopathological features of our cases included
125   patient’s age, presentation symptoms, tumor size and location, uncommon
126   histological features and treatment and follow-up.

127   RESULTS
128   All the patients in our series were of reproductive or perimenopausal age (range, 36-
129   51 years), had an enlarged uterus or a pelvic mass and also had abnormal uterine
130   bleeding. On gross examination, the lesions had an unusual appearance and were
131   often lobulated and irregular with indistinct margins. One of the four cases had no
132   intramural insertion and typically presented as numerous exophytic congested small
133   nodules resembling placental cotyledons. On microscopic examination, tumor
134   described in one of the four cases demonstrated only a cotyledonoid exophytic
135   component with no evident mural dissection. An other case displayed cytologically
136   epithelioid pattern. The two remaining cases were accompanied by limited foci of
137   endometriosis and endosalpingiosis respectively.

138   CONCLUSION
139   The recognition of this benign and unusual appearing variant of leiomyoma by
140   surgeons and pathologists is mandatory in order to avoid overtreatment and preserve
141   the fertility in young women.

142   Highlights

143   ►We    analyse four new cases of cotyledonoid dissecting leiomyoma of the uterus
144   reported in Salah Azaiz Institute department of pathology during the period between
145   2006 and 2011. ► We report clinicopathological features and some histopathological
146   particularities, occasionally causing diagnostic confusion for pathologists who had not
147   experienced such a tumor before► Meticulous histopathological assessment should be
148   performed in order to circumvent misdiagnosis and avoid overtreatment

149   KEYWORDS:        Dissecting   leiomyoma-    Leiomyoma-     Epithelioid-   Endometriosis-
150   Endosalpingiosis
151   INTRODUCTION

152   Conventional leiomyomas are the most frequent neoplasm of the female genital tract. Some
153   variant forms of uterine leiomyoma with distinctive infiltrative growth pattern have been
154   known, including intravenous leiomyomatosis, diffuse leiomyomatosis, leiomyoma with
155   vascular         invasion     and        disseminated        peritoneal      leiomyomatosis.
156   Cotyledonoid dissecting leiomyoma (Sternberg tumor) was proposed for a new form of
157   uterine leiomyoma with histologically benign but distinctive and alarming exophytic placental
158   like mass gross appearance extending from the uterus into the broad ligaments or the pelvic
159   space. This benign smooth muscle tumor with an unusual growth pattern may lead
160   gynecologists and pathologists to false diagnosis and patient overtreatment. For this purpose,
161   this variant of tumor which may create a diagnostic challenge must be well known and kept in
162   mind.
163   We report herein four additional cases of this distinctive benign uterine tumor with unusual
164   growth pattern and some histopathological particularities.

165   METHODS AND RESULTS

166   Four cases of cotyledonoid dissecting leiomyoma are reported in Salah Azaiz Institute
167   department of pathology during the period between 2006 and 2011; the
168   clinicopathologic features of the cases are summarized in Table 1.
169   Case 4 has been previously reported by us in 2008 (1). Patient ages ranged from 47 to
170   52 years (mean: 49.5 years). At laparotomy, three tumors extended from the wall of
171   the uterine body two of them from the serosal side of the uterine fundus and which
172   were characterized by an extrauterine, exophytic growth pattern, multinodular
173   cotyledonoid fungating appearance, and an invasive growth pattern extending from
174   the uterine surface and projecting into the broad ligaments and the pelvic cavity.
175   Macroscopically, the tumors were soft to rubbery and varied from 7 to 30 cm in
176   maximal diameter (mean, 18 cm). The cut surface of the tumors showed white (case
177   2) or deep red (case 1, 3, 4) multilobulated aspect (Figure 1). Tumor described in case
178   3 had no intramural insertion and typically presented as numerous exophytic congested small
179   nodules resembling placental cotyledons
180   An intraoperative frozen section was therefore unlikely to help in identifying the
181   smooth muscle origin of this tumor in cases 3 and 4.
182   Histologically, tumors in cases 1, 2 and 4 had two components, a cotyledonoid
183   exophytic and an intramural component. Both were predominantly formed of
184   disorganized swirls of fascicules of smooth muscle demonstrating an irregularly
185   dissecting growth pattern with elongated processes surrounded by hyaline fibrous
186   matrix (Figure 2). Nuclei were grossly regular with neither mitotic figures nor
187   significant cytological atypia. Coagulative tumor necrosis was also absent. Small
188   congested and dilated clustered veins were identified in the interlobular regions.
189   Tumor described in case 3 demonstrated only a cotyledonoid exophytic component
190   with no evident histological mural dissection. An intravascular intrusion was found
191   only in case 1. These tumors were accompanied by various histological benign
192   lesions. One case (case 4) demonstrated endosalpingiosis in the extrauterine
193   cotyledonoid component, made of numerous glands and cysts lined with a ciliated
194   tubal type epithelium (Figure 3). An other case (case 2) showed limited foci of
195   endometriosis (Figure 4).
    196        Tumor reported in case 3 displayed a cytological epithelioid pattern composed of
    197        sheets of epithelioid cells with finely granular, pale eosinophilic cytoplasm and cental
    198        bland nuclei with no nuclear atypia or mitotic activity (Figure 5).
    199        In the immunohistochemical staining performed in cases 3 and 4, the smooth muscle
    200        tumour cells origin was recognized through positive staining for desmin and
    201        caldesmone. CD34 staining confirmed that there was no intravascular extension o f the
    202        tumor in case 4. Glandular component of endosalpingiosis foci noted in the fourth
    203        case was positive for CK7 and negative for CK20.
    204        Follow- up informations were lacking in case 1 and 2. In case 3, the patient died
    205        during surgery. The patient has gone well with no recurrent disease 10 months after
    206        operation in the fourth case.
Case   Age            Presentation             Location         Size (cm)   Perinodular   Intravascular   Endometriosis or    Tre atment
                                               of tumor                      hydropic        growth        endosalpingiosis
                                                                              change
1         50        Abdominal mass                ---               10         (+)              (+)              ---           Uterine
                                                                                                                                mass
                                                                                                                               excision
2         52        Hypermenorrhea         Posterieor wall of       7          (+)              (-)        Endometriosis         TH
                                                fundus
3         49        Abdominal mass          Serosal side of         30         (+)              (-)              (-)             SH
                                                fundus
4         47      Pelvic mass+vaginal       Serosal side of         25          (-)             (-)       endosalpingiosis     TH,SO
                        bleeding                fundus
    207
    208        TH: Total Hysterectomy   SH: Subtotal Hysterectomy   SO: Salpingo-oophorectpmy

    209        Table 1: Summury of the clincopathologic features

    210
    211
    212        DISCUSSION

    213        Dissecting leiomyoma is an unusual variant of uterine leiomyoma. This term defines the
    214        leiomyomas which causes myometrium dissection. If this tumor exceeds to extrauterine
    215        adnexial area with an exophytic pattern, then this lesion is defined as cotyledonoid dissecting
    216        leiomyoma because of its macroscopic appearance resembling placental cotyledons (4).
    217        However, recently Roth et al had defined cotyledonoid leiomyoma as a new diagnostic term
    218        for benign smooth muscle tumors with an exophytic growth pattern which did not
    219        demonstrate myometrium dissection (4).
    220        Cotyledonoid dissecting leiomyoma (CDL) of the uterus also called “grapelike leiomyoma” is
    221        a very rare tumor among the wide variety of distinctive growth variants described in benign
    222        uterine leiomyoma (2). It is an unusual appearing variant of leiomyoma first described in
    223        1996 by Roth et al. in a series of four cases (3). As they were collected by Dr. Sternberg who
    224        had originally studied the tumor as “a red seaweed lesion”, they were often termed “Sternberg
    225        tumors” (2). Approximatively thirty cases have been reported in the English language
    226        literature (4). CDL of the uterus can be presented in a wide age spectrum of patients ranging
    227        between 23 and 65 years with a mean age of 40, 3 years (4).
    228        The most common clinical presentations consist of pelvic mass and abnormal uterine
    229        bleeding. This variant of leiomyoma is usually large with an average dimension of 17, 7 cm
    230        (4).
231   Macroscopically, it appears that several factors in operation together are required to produce
232   the distinctive gross appearance of cotyledonoid leiomyoma, hence its rarity. First of all, the
233   leiomyoma must originate close to the serosal surface for outward extension into the free
234   space of the pelvic cavity (2). Three of our four reported cases arose in the serosal side of the
235   fundus of uterus. Secondly, the leiomyoma has to exhibit dissecting growth, an uncommon
236   growth pattern in uterine leiomyoma characterized by dissection of the myometrium at the
237   periphery of the tumor in the form of sinuous processes, producing micronodules of swirled
238   neoplastic smooth muscle cells within the myometrium (2). In the only example lacking an
239   intramural dissecting component (case 3), the tumor was located at the interface between the
240   myometrium and the serosa, thus producing exclusively exophytic growth pattern, and the
241   diagnosis in this case was based on the typical macroscopic appearance of numerous
242   exophytic congested small nodules resembling placental cotyledons. Finally, the leiomyoma
243   has to show perinodular hydropic degeneration, another rare form of stromal change in
244   leiomyoma characterized by accumulation of edematous fluid in the connective tissue
245   subdividing the tumor into numerous, small, compact smooth muscle nodules. The hydropic
246   stroma is typically richly vascularized and can even resemble hemangioma. This peculiar
247   appearance can impart an erroneous impression of intravenous leiomyomatosis (2). This
248   hydropic degeneration was documented in cases 1, 2 and 3.
249   The impression of malignancy which may lead to misdiagnosis can be attributed to the
250   alarming fungating appearance, the large size and the appearing widespread infiltrative
251   growth with frequent extension into the pelvic cavity and broad ligament and even the
252   retroperitoneal space, creating a high impression of a malignant process. However, the
253   histological features of this tumor are of mitotically inactive, bland-looking leiomyoma with
254   variably-sized micronodules of disorganized and swirled neoplastic smooth muscle fascicules
255   with a fibrous or edematous typically markedly congested intervening stroma, giving rise to
256   the characteristic red-brown colour of the nodules (2). Marked atypia, mitotic activity and
257   coagulative necrosis are absent (4). This type of tumor is often associated with perinodular
258   hydropic changes (5), endometriosis or endosalpingiosis usually recognized as incidental
259   findings on macroscopic or microscopic examination. Some authors have suggested that
260   frozen sections should be performed to avoid overtreatment and radical surgery during the
261   reproductive age for such a potentially benign lesion (5).
262   Several histologic variants of CDL are described in the literature including cotyledonoid
263   leiomyoma lacking the intramural component, intramural dissecting leiomyoma lacking the
264   extrauterine component, cotyledonoid hydropic intravenous leiomyoma having the
265   characteristics of CDL associated with an additional intravenous component and the
266   epithelioid variant of CDL displaying cytologically epithelioid pattern (6).
267   In summery, CDL are infrequently encountered lesions usually posing a significant
268   management and diagnostic challenge for gynecologists and pathologists with problematic
269   differential diagnoses in microscopic evaluation. Meticulous histopathological assessment
270   should be performed in order to circumvent misdiagnosis and avoid overtreatment.
271   Although macroscopic findings of the tumors seemed to indicate sarcoma, the microscopic
272   findings showed them to be benign.
273   Intra-operatively, the worrisome appearance of the gross specimen is often mistaken for
274   malignant or non-uterine lesions, which may result in overtreatment. Practically and most
275   importantly, intra-operative pathologic consultation (frozen section diagnosis) should be
276   done. Total hysterectomy with close follow-up may be an appropriate treatment.
277
278   Conflict of interest statement:
279   The authors declare that there are no conflicts of interest.

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306   References

307      1) Driss M., Zhioua F., Doghri R., Mrad K., Dhouib R.,Ben Romdhane K., Cotyledonoid
308         dissecting leiomyoma of the uterus associated with endosalpingiosis. Arch Gynecol
309         Obstet (2009) 280:1063–1065.
310      2) Cheuk W., Chan JK C., Liu JYS., Cotyledonoid Leiomyoma :A Benign Ut erine Tumor
311         With Alarming Gross Appearance. Arch Pathol Lab Med. 2002;126:210–213.
312      3) Roth LM, Reed RJ, Sternberg WH. Cotyledonoid dissecting leiomyoma of the uterus:
313         the Sternberg tumor. Am J Surg Pathol 1996; 20: 1455–61.
314      4) Ersِoz S., Turgutalp H.,MunganS.,Güvendİ G.,Güven S. Cotyledonoid Leiomyoma of
315         Uterus: A Case Report. Turk J Path. 2011;27: 257-260.
316      5) Kim MJ.,Park YK.,Cho JH. Cotyledonoid Dissecting Leiomyoma of the Uterus: A
317         Case Report and Review of the Literat ure J Korean Med Sci 2002; 17: 840 -4
318      6) Majd HS.,Ismail L., ·Shaila, Desai SA, Reginald PW. Epithelioid cotyledonoid
319         dissecting leiomyoma: a case report and review of the literature. Arch Gynecol Obstet
320         (2011) 283:771–774
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348   Legends
349   Figure 1: The tumor formed deep red, multiple nodules wrapped by anastomosing vascular
350   network resembling placental cotyledons, extending to large ligaments.
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352   Figure 2: Multiple micronodules of smooth muscle dissecting the myometrium with swirling
353   growth pattern (inset), abundant congested blood vessels, and perinodular hydropic
354   degeneration (inset). (hematoxylin-eosin X 20, inset: hematoxytin-eosin X 40).
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356   Figure 3: Micronodules of smooth muscle within the myometrium adjacent to
357   multiple limited foci of endosalpingiosis made of variously sized glands and cysts lined
358   by benign-appearing epithelium in case 4 (hematoxylin-eosin, X20).
359   Inset: Lining epithelium showed a stratification of ciliated columnar cells (hematoxylin-
360   eosin, X40).
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362   Figure 4: Smooth muscle micronodules dissecting the myometrium wih foci of
363   endometriosis in case 2. (hematoxylin-eosin, X10).
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366   Figure 5: cytologically epithelioid pattern described in case 3 (hematoxylin-eosin, X20,
367   inset: hematoxylin-eosin X 40).
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370   Figure 6: Positive immunostaining of smooth muscle tumor cells with Desmine and
371   Caldesmone (Inset)
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