ª Springer Science+Business Media New York 2015
Abdominal Imaging
Abdom Imaging (2015) DOI: 10.1007/s00261-015-0486-9
Case report: MR imaging features of disseminated uterine leiomyosarcoma presenting after hysterectomy with morcellation Tadi Ciszak,1 Pardeep K. Mittal,1 Patrick Sullivan,2 Kenneth Cardona,2 Krisztina Z. Hanley,3 Namita Khanna,4 Courtney Coursey Moreno1 1
Department of Radiology & Imaging Sciences, Emory University School of Medicine, Atlanta, GA, USA Division of Surgical Oncology, Department of Surgery, Winship Cancer Institute, Emory University, Atlanta, GA, USA 3 Department of Pathology, Emory University School of Medicine, Atlanta, GA, USA 4 Division of Gynecologic Oncology, Department of Gynecology and Obstetrics, Emory University School of Medicine, Atlanta, GA, USA 2
Abstract A 53-year-old woman underwent elective hysterectomy for symptomatic anemia secondary to abnormal uterine bleeding. She presented 15 months later with complaints of abdominal fullness. Abdominopelvic magnetic resonance imaging demonstrated multiple confluent enhancing solid masses centered in the pelvis and extending cranially to the level of the umbilicus. Additional separate nodules also were visible along the peritoneum. Biopsy demonstrated leiomyosarcoma. Additional clinical information was obtained, which revealed that the patient’s prior hysterectomy was performed with morcellation. In November 2014, the United States Food and Drug Administration issued a warning discouraging the use of morcellation during hysterectomy and myomectomy because of the risk of seeding unsuspected malignancy. Radiologists should be aware of this potential complication of morcellation and its imaging appearance so that the correct diagnosis can be suggested in the imaging report. Key words: Leiomyosarcoma—Morcellation—Magnetic resonance imaging—Leiomyoma
enough to be removed through a port site [1, 2]. Intra abdominal spread of uterine malignancy, benign fibroids, adenomyosis, and trophoblastic tissue by morcellation has been reported [1, 3–6]. Concerns regarding the potential for disseminating unsuspected malignant tissue in the peritoneal cavity as a result of morcellation prompted the United States Food and Drug Administration (FDA) to issue a warning in November 2014 discouraging the use of power morcellation during hysterectomy and myomectomy [7]. The reported risk of unsuspected sarcomatous change in the uterus at time of hysterectomy or myomectomy ranges from 1 in 204 to 1 in 1788 [1, 8]. We report MR imaging features of a case of disseminated uterine leiomyosarcoma presenting 15 months following elective hysterectomy with morcellation. Surgical pathology at time of hysterectomy at an outside institution demonstrated benign uterine fibroids and adenomyosis. It is important for radiologists to be aware of this complication of morcellation and the imaging features of disseminated uterine malignancy so that the correct diagnosis can be suggested in the imaging report.
Case report Laparoscopic uterine power morcellation is utilized during myomectomy and hysterectomy for benign disease. The morcellator is a device with a fast rotating cylindrical knife that cuts tissue into fragments small
Correspondence to: Courtney Coursey Moreno; email: courtney. [email protected]
A 53-year-old woman with a past medical history of abnormal uterine bleeding underwent elective hysterectomy for symptomatic anemia. Preoperative ultrasound performed at an outside institution demonstrated an enlarged uterus measuring 13 9 7 9 11 cm with multiple fibroids, the largest measuring 6 9 5 9 6 cm. Endometrial biopsy was attempted at an outside institution but could not be performed due to inability to pass a pipelle
T. Ciszak et al.: Case report: MR imaging features of disseminated uterine leiomyosarcoma
through the cervix. The preoperative surgical diagnosis was ‘‘large uterine myoma’’, and the surgeon described an enlarged uterus measuring approximately 16– 17 weeks with multiple nodular fibroids in the operative report. A morcellator was used during the surgery so that the uterus could be cut into fragments that were small enough to remove through port sites. The surgeon’s postoperative diagnosis was also ‘‘large uterine myoma’’. Surgical pathology at an outside institution demonstrated innumerable pieces of a morcellated hysterectomy specimen. The tissue fragments were interpreted as benign leiomyomata and adenomyosis. No nuclear atypia, mitotic activity, and or malignant features were seen. Beginning approximately 15 months after the hysterectomy, the patient began to experience symptoms of pressure on her bladder and bloating. Initial workup at an outside institution included a colonoscopy which demonstrated a few small benign polyps but was otherwise normal. Approximately 18 months after the hysterectomy, the patient underwent CT imaging of the abdomen and pelvis to evaluate her symptoms. The CT scan revealed a large mass filling the pelvis and extending into the abdomen with tumor extension into the umbil-
Fig. 1. A Sagittal reformation from a contrast-enhanced CT scan demonstrates an enhancing solid mass located along the cranial margin of the cervix (black arrow) and extending above the level of the umbilicus. Abnormal soft tissue also extends into the umbilical port site (white arrow). B Axial
Fig. 2. A Sagittal T2-weighted MR demonstrates a low-sig- c nal mass (asterisk) located posterior to the urinary bladder (solid white arrow), anterior to the rectum (dashed white arrow), and along the cranial margin of the cervix (black arrow). The dominant mass is separated from the cervix by a small amount of fluid. B Axial T2-weighted image demonstrates portions of the mass (asterisk) to be primarily low signal with relatively small areas of internal cystic change (solid arrow). A separate low-signal tumoral implant is located along the peritoneal lining in the left lower quadrant (dashed arrow). C Axial T1-weighted precontrast image demonstrates predominantly intermediate signal intensity within the mass (asterisk). D 60-sec post-contrast T1-weighted image demonstrates areas of homogeneous enhancement in portions of the dominant mass (asterisk) and in the satellite lesion (dashed arrow) along with areas of heterogeneous enhancement (solid arrow). E 3-min delayed post-contrast T1-weighted image demonstrates persistent homogeneous enhancement in the dominant mass (asterisk) and satellite nodule (arrow) without washout.
ical port site (Fig. 1). Additional separate peritoneal nodules were also visible. Magnetic resonance imaging (MRI) was obtained for further characterization including to evaluate the rela-
portal venous phase contrast-enhanced CT image demonstrates heterogeneous enhancement within the mass (asterisk). The mass is located posterior to the urinary bladder (white arrow).
T. Ciszak et al.: Case report: MR imaging features of disseminated uterine leiomyosarcoma
T. Ciszak et al.: Case report: MR imaging features of disseminated uterine leiomyosarcoma
Fig. 3. A Core biopsy from the soft tissue mass indicated on imaging above. Fascicles of spindled cells intermixed with rare large abnormal cells (hematoxylin eosin stain, 9100 magnification). B High power view of the core biopsy
demonstrates cytologically atypical cells with areas of necrosis in the lower corner (hematoxylin eosin stain, 9400 magnification).
tionship of the tumors to surrounding structures such as the cervix. MRI also demonstrated a large, lobulated mass centered within the pelvis (Fig. 2). The mass did not appear to be arising from the cervix, and the cervix appeared normal (Fig. 2). Portions of the mass demonstrated homogeneous low T2 signal, intermediate T1 signal, and avid homogeneous enhancement that persisted at delayed phase imaging (Fig. 2). Other portions of the dominant mass appeared more heterogeneous with areas of cystic necrosis and heterogeneous enhancement. Several separate discrete tumors were also visible along the peritoneum. Biopsy revealed malignant spindle cells in a background of tumor necrosis (Fig. 3). Immunohistochemical stains were supportive of a diagnosis of leiomyosarcoma. Morcellation performed during the hysterectomy at the outside institution likely resulted in either seeding the patient’s peritoneal cavity with an undiagnosed leiomyosarcoma or seeding the peritoneal cavity with benign myometrial tissue that subsequently underwent malignant degeneration. That the cervical remnant appeared normal and separate from the pelvic masses on MR ruled out a cervical remnant origin of the pelvic mass.
coma preoperatively, although sarcomas may appear more heterogeneous and necrotic as compared to benign fibroids at MRI [9–11]. The workup of an individual with suspected fibroids may include imaging to define the size, location, and number of fibroids. Depending on fibroid characteristics, the patient may be offered myomectomy, hysterectomy, or uterine artery embolization. Morcellation is performed during laparoscopic hysterectomy or myomectomy so that tissue fragments can be removed through a port site. Advantages of such minimally invasive gynecological surgery include less post-operative pain, decreased risk of surgical site infection, and faster healing and recovery as compared to an open hysterectomy or myomectomy [12]. Despite the 2014 FDA warning, morcellation is still performed at some centers after a discussion of risks and benefits with the patient [13]. Historically, morcellation has been performed with an open technique. With open technique, small tissue fragments may be dispersed and remain within the peritoneal cavity. Techniques for contained morcellation (example, within an endoscopy bag) have been described more recently [14]. All tissue removed at time of hysterectomy typically is sent for pathologic analysis. Gross examination of morcellated hysterectomy tissue can be challenging because tissue is fragmented and anatomic landmarks for specimen orientation are lost [15, 16]. It is also difficult to grossly assess the cut surface of fibroids due to fragmentation. General recommendations for examination of uterine smooth muscle tumors include targeted sampling of areas of necrosis, hemorrhage, or other abnormalities. In a morcellated specimen with small tissue fragments, these suspicious areas easily can be overlooked and unsampled.
Discussion When issuing its 2014 warning discouraging the use of open power morcellation, the FDA cited the potential for unsuspected uterine sarcoma at time of hysterectomy or myomectomy, the lack of a method to reliably identify uterine sarcomas preoperatively, and the poor prognosis associated with disseminated uterine malignancy as the reasons for its warning [7]. Neither MRI nor ultrasound can reliably distinguish leiomyoma from leiomyosar-
T. Ciszak et al.: Case report: MR imaging features of disseminated uterine leiomyosarcoma
If unsuspected malignant or atypical cells are found at histology, the patient may undergo an exploratory open surgery to remove any residual tumor, adjuvant chemotherapy may be offered based on pathology, or a more conservative approach with serial imaging surveillance may be taken [6]. Prior case reports have described individuals presenting 6–14 months after initial hysterectomy with morcellation with solitary or multifocal uterine malignancies [17, 18]. Whether the malignant cells were disseminated at time of morcellation or benign disseminated cells underwent malignant degeneration cannot be determined in the present case. An important imaging characteristic is disease topography as morcellated uterine tissue is mechanically dispersed during surgery [7]. An additional clue to the diagnosis may be the presence of tumor in a port site (Fig. 1), a potential site for implantation as occult disease or surgical instruments with cellular deposits are removed from the patient. Morcellated tissue is typically removed through lateral port sites during a hysterectomy, and the viewing scope is typically placed through the umbilical port site. Differential diagnosis based on MR imaging is primarily a multifocal sarcoma given avid post-contrast enhancement without washout. Multiple benign leiomyomas could have a similar appearance although tumoral heterogeneity is suspicious for sarcomatous degeneration. Leiomyosarcomas may appear heterogeneous with areas of cystic necrosis [19]. Leiomyomas and leiomyosarcomas may both demonstrate areas of intermediate and low T2 signal intensity. There is also overlap in the appearance of leiomyomas and leiomyosarcomas at diffusion-weighted imaging [20]. Leiomyosarcomas also cannot be reliably distinguished from benign leiomyomas at ultrasound [11, 12]. For example, in a small study of 8 patients with leiomyosarcomas and 21 patients with leiomyomas, increased central and peripheral vascularity was found to be 100% sensitive for leiomyosarcoma but to have a positive predictive value of only 19% [11]. There may be a future role for nuclear medicine studies in the differentiation of leiomyomas from leiomyosarcomas. In one study, patients with uterine sarcomas showed higher uptake of fluorine 18 (18F) fluorodeoxyglucose and a significantly lower uptake of 16-a-[18F]fluoro-17b-estradiaol as compared to individuals with leiomyomas [21]. Reliable specific uptake value thresholds for the differentiation of leiomyomas and leiomyosarcomas remain to be established and validated. Other differential diagnostic considerations based on imaging include gastrointestinal stromal tumor, metastatic disease, sarcoma from other origin, or ovarian tumors if the ovaries were not removed. Tissue sampling was ultimately required to establish a diagnosis in the present case.
Past surgical history of hysterectomy with benign disease found at surgical pathology should not dissuade the radiologist from suggesting the possibility of a sarcoma of uterine origin as sarcomatous elements may not be identified at the time of initial surgery as in the above case. The differential diagnosis at gross pathology of multiple peritoneal nodules in the setting of prior hysterectomy performed with morcellation includes two entities: disseminated peritoneal leiomyomatosis (DPL) and metastatic leiomyosarcoma. DPL is characterized by proliferation of benign smooth muscle cells on peritoneal surfaces. Grossly and histologically these tumors resemble tiny leiomyomas. These tumors usually clinically behave in a benign fashion, but rare cases have transformed into leiomyosarcoma [22, 23]. Metastatic leiomyosarcoma is diagnosed based on the mitotic count and with use of immunohistochemical stains including smooth muscle actin and desmin. Treatment options for individuals with disseminated uterine leiomyosarcoma include tumor debulking and chemotherapy. Prognosis is generally poor. The overall 5-year survival rate for individuals with unsuspected leiomyosarcoma who underwent morcellation is 46% [24]. Prognosis is also poor for uterine leiomyosarcomas even without morcellation with a similar overall 5-year survival of 44–45% [25, 26]. In conclusion, we present a case of disseminated uterine leiomyosarcoma presenting 15 months after elective hysterectomy with morcellation with surgical pathology demonstrating benign disease at time of hysterectomy. Radiologists should be aware of this potential complication of morcellation and its imaging appearance so that the correct diagnosis can be suggested in the imaging report.
References 1. Brolmann H, Tanos V, Grimbizis G, et al. (2015) Options on fibroid morcellation: a literature review. Gynecol Surg 12:13–15 2. Steiner RA, Wight E, Tadir Y, Haller U (1993) Electrical cutting device for laparoscopic removal of tissue from the abdominal cavity. Obstet Gynecol 81:471–474 3. Nezhat C, Kho K (2010) Iatrogenic myomas: new class of myomas? J Minim Invasive Gynecol 17:544–550 4. Holloran-Schwartz MB, Fierro M, Tritto A (2015) Delayed presentation of a paracytic myoma fragment after laparoscopic supracervical hysterectomy requiring small bowel resection. A case report. J Reprod Med 60:75–77 5. Pereira N, Buchanan TR, Wishall KM, et al. (2015) Electric morcellation-related reoperations after laparoscopic myomectomy and nonmyomectomy procedures. J Min Invasive Gynecology 22:163– 176 6. Seidman MA, Oduyebo T, Muto MG, et al. (2012) Peritoneal dissemination complicating morcellation of uterine mesenchymal neoplasms. PLoS One 7:e50058. doi:10.1371/journal.pone.0050058 7. http://www.fda.gov/MedicalDevices/Safety/AlertsandNotices/ucm 393576.htm Revised 24 Nov 2014. Accessed 21 Mar 2015. 8. Hamptom T (2014) Critics of fibroid removal procedure question risks it may pose for women with undetected uterine cancer. JAMA 311:891–893 9. Sala E, Rockall AG, Freeman SJ, Mitchell DG, Reinhold C (2013) The added role of MR imaging in treatment stratification of pa-
T. Ciszak et al.: Case report: MR imaging features of disseminated uterine leiomyosarcoma
10. 11. 12.
13. 14. 15. 16. 17.
18.
tients with gynecologic malignancies: what the radiologist needs to know. Radiology 266:717–740 Aviram R, Ochshorn Y, Markovitch O, et al. (2005) Uterine sarcomas versus leiomyomas: gray-scale and Doppler sonographic findings. J Clin Ultrasound 33:10–13 Exacoustos C, Romanini MA, Amadio A, et al. (2007) Can grayscale and color Doppler sonography differentiate between uterine leiomyosarcoma and leiomyoma? J Clin Ultrasound 25:449–457 Kluivers KB, Hendriks JCM, Mol BWJ, et al. (2007) Quality of life and surgical outcome after total laparoscopic hysterectomy versus total abdominal hysterectomy for benign disease: a randomized, controlled trial. J Minim Invasive Gynecol 14:145–152 Ton R, Kilic GS, Phelps JY (2015) A medical-legal review of power morcellation in the face of the recent FDA warning and litigation. J Minim Invasive Gynecol 22:564–572 Srouji SS, Kaser DJ, Garigulo AR (2015) Techniques for contained morcellation in gynecologic surgery. Fertil Steril 103:e43 Ehdaivand S, Simon RA, Sung CJ, et al. (2014) Incidental gynecologic neoplasms in morcellated uterine specimens: a case series with follow-up. Hum Pathol 45:2311–2317 Rivard C, Salhadar A, Kenton K (2012) New challenges in detecting, grading, and staging endometrial cancer after uterine morcellation. J Minim Invasive Gynecol 19:313–316 Turner T, Secord AA, Lowery WJ, Sfakianos G, Lee PS (2013) Metastatic adenocarcinoma after laparoscopic supracervical hysterectomy with morcellation. A case report. Gynecol Oncol Case Rep 5:19–21 Rekha W, Amita M, Sudeep G, Hemant T (2005) Unexpected complication of uterine myoma morcellation. Aust N Z J Obstet Gynaecol 45:248–249
19. Sala E, Wakely S, Senior E, Lomas D (2007) MRI of malignant neoplasms of the uterine corpus and cervix. AJR Am J Roentgenol 188:1577–1587 20. Saremi F, Knoll AN, Bendavid OJ, et al. (2009) Characterization of genitourinary lesions with diffusion-weighted imaging. Radiographics 29:1295–1317 21. Tsujikawa T, Yoshida T, Mori T, et al. (2008) Uterine tumors: pathophysiologic imaging with 16alpha-[18F]fluoro-17beta-estradiol and 18F fluorodeoxyglucose PET-initial experience. Radiology 248:599–605 22. Kumar S, Sharma JB, Verma D, et al. (2008) Disseminated peritoneal leiomyomatosis: an unusual complication of laparoscopic myomectomy. Arch Gynecol Obstet 278:93–95 23. Miyake T, Enomoto T, Ueda Y, et al. (2009) A case of disseminated peritoneal leiomyomatosis developing after laparoscopic-assisted myomectomy. Gynecol Obstet Invest 67:96–102 24. Park JY, Park SK, Kim DY, et al. (2011) The impact of tumor morcellation during surgery on the prognosis of patients with apparently early uterine leiomyosarcoma. Gynecol Oncol 122:255– 259 25. Dinh TA, Oliva EA, Fuller AF Jr, Lee H, Goodman A (2004) The treatment of uterine leiomyosarcoma. Results from a 10-year experience (1990-1999) at the Massachusetts General Hospital. Gynecol Oncol 92:648–652 26. Tasci T, Karalok A, Taskin S, et al. (2015) Does lymphadenectomy improve survival in uterine leiomyosarcoma? Int J Gynecol Cancer. doi:10.1097/IGC.0000000000000452
Abdominal Imaging
Abdom Imaging (2015) DOI: 10.1007/s00261-015-0486-9
Case report: MR imaging features of disseminated uterine leiomyosarcoma presenting after hysterectomy with morcellation Tadi Ciszak,1 Pardeep K. Mittal,1 Patrick Sullivan,2 Kenneth Cardona,2 Krisztina Z. Hanley,3 Namita Khanna,4 Courtney Coursey Moreno1 1
Department of Radiology & Imaging Sciences, Emory University School of Medicine, Atlanta, GA, USA Division of Surgical Oncology, Department of Surgery, Winship Cancer Institute, Emory University, Atlanta, GA, USA 3 Department of Pathology, Emory University School of Medicine, Atlanta, GA, USA 4 Division of Gynecologic Oncology, Department of Gynecology and Obstetrics, Emory University School of Medicine, Atlanta, GA, USA 2
Abstract A 53-year-old woman underwent elective hysterectomy for symptomatic anemia secondary to abnormal uterine bleeding. She presented 15 months later with complaints of abdominal fullness. Abdominopelvic magnetic resonance imaging demonstrated multiple confluent enhancing solid masses centered in the pelvis and extending cranially to the level of the umbilicus. Additional separate nodules also were visible along the peritoneum. Biopsy demonstrated leiomyosarcoma. Additional clinical information was obtained, which revealed that the patient’s prior hysterectomy was performed with morcellation. In November 2014, the United States Food and Drug Administration issued a warning discouraging the use of morcellation during hysterectomy and myomectomy because of the risk of seeding unsuspected malignancy. Radiologists should be aware of this potential complication of morcellation and its imaging appearance so that the correct diagnosis can be suggested in the imaging report. Key words: Leiomyosarcoma—Morcellation—Magnetic resonance imaging—Leiomyoma
enough to be removed through a port site [1, 2]. Intra abdominal spread of uterine malignancy, benign fibroids, adenomyosis, and trophoblastic tissue by morcellation has been reported [1, 3–6]. Concerns regarding the potential for disseminating unsuspected malignant tissue in the peritoneal cavity as a result of morcellation prompted the United States Food and Drug Administration (FDA) to issue a warning in November 2014 discouraging the use of power morcellation during hysterectomy and myomectomy [7]. The reported risk of unsuspected sarcomatous change in the uterus at time of hysterectomy or myomectomy ranges from 1 in 204 to 1 in 1788 [1, 8]. We report MR imaging features of a case of disseminated uterine leiomyosarcoma presenting 15 months following elective hysterectomy with morcellation. Surgical pathology at time of hysterectomy at an outside institution demonstrated benign uterine fibroids and adenomyosis. It is important for radiologists to be aware of this complication of morcellation and the imaging features of disseminated uterine malignancy so that the correct diagnosis can be suggested in the imaging report.
Case report Laparoscopic uterine power morcellation is utilized during myomectomy and hysterectomy for benign disease. The morcellator is a device with a fast rotating cylindrical knife that cuts tissue into fragments small
Correspondence to: Courtney Coursey Moreno; email: courtney. [email protected]
A 53-year-old woman with a past medical history of abnormal uterine bleeding underwent elective hysterectomy for symptomatic anemia. Preoperative ultrasound performed at an outside institution demonstrated an enlarged uterus measuring 13 9 7 9 11 cm with multiple fibroids, the largest measuring 6 9 5 9 6 cm. Endometrial biopsy was attempted at an outside institution but could not be performed due to inability to pass a pipelle
T. Ciszak et al.: Case report: MR imaging features of disseminated uterine leiomyosarcoma
through the cervix. The preoperative surgical diagnosis was ‘‘large uterine myoma’’, and the surgeon described an enlarged uterus measuring approximately 16– 17 weeks with multiple nodular fibroids in the operative report. A morcellator was used during the surgery so that the uterus could be cut into fragments that were small enough to remove through port sites. The surgeon’s postoperative diagnosis was also ‘‘large uterine myoma’’. Surgical pathology at an outside institution demonstrated innumerable pieces of a morcellated hysterectomy specimen. The tissue fragments were interpreted as benign leiomyomata and adenomyosis. No nuclear atypia, mitotic activity, and or malignant features were seen. Beginning approximately 15 months after the hysterectomy, the patient began to experience symptoms of pressure on her bladder and bloating. Initial workup at an outside institution included a colonoscopy which demonstrated a few small benign polyps but was otherwise normal. Approximately 18 months after the hysterectomy, the patient underwent CT imaging of the abdomen and pelvis to evaluate her symptoms. The CT scan revealed a large mass filling the pelvis and extending into the abdomen with tumor extension into the umbil-
Fig. 1. A Sagittal reformation from a contrast-enhanced CT scan demonstrates an enhancing solid mass located along the cranial margin of the cervix (black arrow) and extending above the level of the umbilicus. Abnormal soft tissue also extends into the umbilical port site (white arrow). B Axial
Fig. 2. A Sagittal T2-weighted MR demonstrates a low-sig- c nal mass (asterisk) located posterior to the urinary bladder (solid white arrow), anterior to the rectum (dashed white arrow), and along the cranial margin of the cervix (black arrow). The dominant mass is separated from the cervix by a small amount of fluid. B Axial T2-weighted image demonstrates portions of the mass (asterisk) to be primarily low signal with relatively small areas of internal cystic change (solid arrow). A separate low-signal tumoral implant is located along the peritoneal lining in the left lower quadrant (dashed arrow). C Axial T1-weighted precontrast image demonstrates predominantly intermediate signal intensity within the mass (asterisk). D 60-sec post-contrast T1-weighted image demonstrates areas of homogeneous enhancement in portions of the dominant mass (asterisk) and in the satellite lesion (dashed arrow) along with areas of heterogeneous enhancement (solid arrow). E 3-min delayed post-contrast T1-weighted image demonstrates persistent homogeneous enhancement in the dominant mass (asterisk) and satellite nodule (arrow) without washout.
ical port site (Fig. 1). Additional separate peritoneal nodules were also visible. Magnetic resonance imaging (MRI) was obtained for further characterization including to evaluate the rela-
portal venous phase contrast-enhanced CT image demonstrates heterogeneous enhancement within the mass (asterisk). The mass is located posterior to the urinary bladder (white arrow).
T. Ciszak et al.: Case report: MR imaging features of disseminated uterine leiomyosarcoma
T. Ciszak et al.: Case report: MR imaging features of disseminated uterine leiomyosarcoma
Fig. 3. A Core biopsy from the soft tissue mass indicated on imaging above. Fascicles of spindled cells intermixed with rare large abnormal cells (hematoxylin eosin stain, 9100 magnification). B High power view of the core biopsy
demonstrates cytologically atypical cells with areas of necrosis in the lower corner (hematoxylin eosin stain, 9400 magnification).
tionship of the tumors to surrounding structures such as the cervix. MRI also demonstrated a large, lobulated mass centered within the pelvis (Fig. 2). The mass did not appear to be arising from the cervix, and the cervix appeared normal (Fig. 2). Portions of the mass demonstrated homogeneous low T2 signal, intermediate T1 signal, and avid homogeneous enhancement that persisted at delayed phase imaging (Fig. 2). Other portions of the dominant mass appeared more heterogeneous with areas of cystic necrosis and heterogeneous enhancement. Several separate discrete tumors were also visible along the peritoneum. Biopsy revealed malignant spindle cells in a background of tumor necrosis (Fig. 3). Immunohistochemical stains were supportive of a diagnosis of leiomyosarcoma. Morcellation performed during the hysterectomy at the outside institution likely resulted in either seeding the patient’s peritoneal cavity with an undiagnosed leiomyosarcoma or seeding the peritoneal cavity with benign myometrial tissue that subsequently underwent malignant degeneration. That the cervical remnant appeared normal and separate from the pelvic masses on MR ruled out a cervical remnant origin of the pelvic mass.
coma preoperatively, although sarcomas may appear more heterogeneous and necrotic as compared to benign fibroids at MRI [9–11]. The workup of an individual with suspected fibroids may include imaging to define the size, location, and number of fibroids. Depending on fibroid characteristics, the patient may be offered myomectomy, hysterectomy, or uterine artery embolization. Morcellation is performed during laparoscopic hysterectomy or myomectomy so that tissue fragments can be removed through a port site. Advantages of such minimally invasive gynecological surgery include less post-operative pain, decreased risk of surgical site infection, and faster healing and recovery as compared to an open hysterectomy or myomectomy [12]. Despite the 2014 FDA warning, morcellation is still performed at some centers after a discussion of risks and benefits with the patient [13]. Historically, morcellation has been performed with an open technique. With open technique, small tissue fragments may be dispersed and remain within the peritoneal cavity. Techniques for contained morcellation (example, within an endoscopy bag) have been described more recently [14]. All tissue removed at time of hysterectomy typically is sent for pathologic analysis. Gross examination of morcellated hysterectomy tissue can be challenging because tissue is fragmented and anatomic landmarks for specimen orientation are lost [15, 16]. It is also difficult to grossly assess the cut surface of fibroids due to fragmentation. General recommendations for examination of uterine smooth muscle tumors include targeted sampling of areas of necrosis, hemorrhage, or other abnormalities. In a morcellated specimen with small tissue fragments, these suspicious areas easily can be overlooked and unsampled.
Discussion When issuing its 2014 warning discouraging the use of open power morcellation, the FDA cited the potential for unsuspected uterine sarcoma at time of hysterectomy or myomectomy, the lack of a method to reliably identify uterine sarcomas preoperatively, and the poor prognosis associated with disseminated uterine malignancy as the reasons for its warning [7]. Neither MRI nor ultrasound can reliably distinguish leiomyoma from leiomyosar-
T. Ciszak et al.: Case report: MR imaging features of disseminated uterine leiomyosarcoma
If unsuspected malignant or atypical cells are found at histology, the patient may undergo an exploratory open surgery to remove any residual tumor, adjuvant chemotherapy may be offered based on pathology, or a more conservative approach with serial imaging surveillance may be taken [6]. Prior case reports have described individuals presenting 6–14 months after initial hysterectomy with morcellation with solitary or multifocal uterine malignancies [17, 18]. Whether the malignant cells were disseminated at time of morcellation or benign disseminated cells underwent malignant degeneration cannot be determined in the present case. An important imaging characteristic is disease topography as morcellated uterine tissue is mechanically dispersed during surgery [7]. An additional clue to the diagnosis may be the presence of tumor in a port site (Fig. 1), a potential site for implantation as occult disease or surgical instruments with cellular deposits are removed from the patient. Morcellated tissue is typically removed through lateral port sites during a hysterectomy, and the viewing scope is typically placed through the umbilical port site. Differential diagnosis based on MR imaging is primarily a multifocal sarcoma given avid post-contrast enhancement without washout. Multiple benign leiomyomas could have a similar appearance although tumoral heterogeneity is suspicious for sarcomatous degeneration. Leiomyosarcomas may appear heterogeneous with areas of cystic necrosis [19]. Leiomyomas and leiomyosarcomas may both demonstrate areas of intermediate and low T2 signal intensity. There is also overlap in the appearance of leiomyomas and leiomyosarcomas at diffusion-weighted imaging [20]. Leiomyosarcomas also cannot be reliably distinguished from benign leiomyomas at ultrasound [11, 12]. For example, in a small study of 8 patients with leiomyosarcomas and 21 patients with leiomyomas, increased central and peripheral vascularity was found to be 100% sensitive for leiomyosarcoma but to have a positive predictive value of only 19% [11]. There may be a future role for nuclear medicine studies in the differentiation of leiomyomas from leiomyosarcomas. In one study, patients with uterine sarcomas showed higher uptake of fluorine 18 (18F) fluorodeoxyglucose and a significantly lower uptake of 16-a-[18F]fluoro-17b-estradiaol as compared to individuals with leiomyomas [21]. Reliable specific uptake value thresholds for the differentiation of leiomyomas and leiomyosarcomas remain to be established and validated. Other differential diagnostic considerations based on imaging include gastrointestinal stromal tumor, metastatic disease, sarcoma from other origin, or ovarian tumors if the ovaries were not removed. Tissue sampling was ultimately required to establish a diagnosis in the present case.
Past surgical history of hysterectomy with benign disease found at surgical pathology should not dissuade the radiologist from suggesting the possibility of a sarcoma of uterine origin as sarcomatous elements may not be identified at the time of initial surgery as in the above case. The differential diagnosis at gross pathology of multiple peritoneal nodules in the setting of prior hysterectomy performed with morcellation includes two entities: disseminated peritoneal leiomyomatosis (DPL) and metastatic leiomyosarcoma. DPL is characterized by proliferation of benign smooth muscle cells on peritoneal surfaces. Grossly and histologically these tumors resemble tiny leiomyomas. These tumors usually clinically behave in a benign fashion, but rare cases have transformed into leiomyosarcoma [22, 23]. Metastatic leiomyosarcoma is diagnosed based on the mitotic count and with use of immunohistochemical stains including smooth muscle actin and desmin. Treatment options for individuals with disseminated uterine leiomyosarcoma include tumor debulking and chemotherapy. Prognosis is generally poor. The overall 5-year survival rate for individuals with unsuspected leiomyosarcoma who underwent morcellation is 46% [24]. Prognosis is also poor for uterine leiomyosarcomas even without morcellation with a similar overall 5-year survival of 44–45% [25, 26]. In conclusion, we present a case of disseminated uterine leiomyosarcoma presenting 15 months after elective hysterectomy with morcellation with surgical pathology demonstrating benign disease at time of hysterectomy. Radiologists should be aware of this potential complication of morcellation and its imaging appearance so that the correct diagnosis can be suggested in the imaging report.
References 1. Brolmann H, Tanos V, Grimbizis G, et al. (2015) Options on fibroid morcellation: a literature review. Gynecol Surg 12:13–15 2. Steiner RA, Wight E, Tadir Y, Haller U (1993) Electrical cutting device for laparoscopic removal of tissue from the abdominal cavity. Obstet Gynecol 81:471–474 3. Nezhat C, Kho K (2010) Iatrogenic myomas: new class of myomas? J Minim Invasive Gynecol 17:544–550 4. Holloran-Schwartz MB, Fierro M, Tritto A (2015) Delayed presentation of a paracytic myoma fragment after laparoscopic supracervical hysterectomy requiring small bowel resection. A case report. J Reprod Med 60:75–77 5. Pereira N, Buchanan TR, Wishall KM, et al. (2015) Electric morcellation-related reoperations after laparoscopic myomectomy and nonmyomectomy procedures. J Min Invasive Gynecology 22:163– 176 6. Seidman MA, Oduyebo T, Muto MG, et al. (2012) Peritoneal dissemination complicating morcellation of uterine mesenchymal neoplasms. PLoS One 7:e50058. doi:10.1371/journal.pone.0050058 7. http://www.fda.gov/MedicalDevices/Safety/AlertsandNotices/ucm 393576.htm Revised 24 Nov 2014. Accessed 21 Mar 2015. 8. Hamptom T (2014) Critics of fibroid removal procedure question risks it may pose for women with undetected uterine cancer. JAMA 311:891–893 9. Sala E, Rockall AG, Freeman SJ, Mitchell DG, Reinhold C (2013) The added role of MR imaging in treatment stratification of pa-
T. Ciszak et al.: Case report: MR imaging features of disseminated uterine leiomyosarcoma
10. 11. 12.
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