Indian Journal of Pathology and Microbiology
Home About us Instructions Submission Subscribe Advertise Contact e-Alerts Ahead Of Print Login 
Users Online: 1907
Print this page  Email this page Bookmark this page Small font sizeDefault font sizeIncrease font size

  Table of Contents    
Year : 2014  |  Volume : 57  |  Issue : 3  |  Page : 447-449
Primary extrauterine endometrial stromal sarcoma: Located in pelvic and abdominal tissue and arising in endometriosis

1 Department of Pathology, Tata Medical Center, Kolkata, India
2 Department of Gynaecology, Tata Medical Center, Kolkata, India

Click here for correspondence address and email

Date of Web Publication14-Aug-2014


Primary extrauterine endometrial stromal sarcoma is a rare tumor and it is infrequently associated with endometriosis. We are reporting a case of this unusual tumor in a 42-year-old female who presented with multiple nodules of tumor in the abdomen and pelvis and with metastases in para-aortic lymph nodes. The right parametrium, in addition, had a focus of endometriosis, which was contiguous with the tumor, confirming its origin.

Keywords: Extrauterine endometrial stromal sarcoma

How to cite this article:
Ghosal T, Roy A, Kurian S. Primary extrauterine endometrial stromal sarcoma: Located in pelvic and abdominal tissue and arising in endometriosis. Indian J Pathol Microbiol 2014;57:447-9

How to cite this URL:
Ghosal T, Roy A, Kurian S. Primary extrauterine endometrial stromal sarcoma: Located in pelvic and abdominal tissue and arising in endometriosis. Indian J Pathol Microbiol [serial online] 2014 [cited 2022 Oct 7];57:447-9. Available from:

   Introduction Top

Primary extrauterine endometrial stromal sarcoma (ESS) is an unusual tumor and only about 80 cases have been reported thus far in literature. [1],[2] The largest series reported was from the M D Anderson Cancer Center, Texas, in 2013. That was a case series of 63 cases collected over a period of 21 years. [1] In that series the most common site of involvement was the abdominal peritoneum, followed by the bowel wall, the ovaries, and the pelvis. Half of those cases were associated with endometriosis.

Most case reports however are those of ESS of the bowel wall, some of which are associated endometriosis. [2],[3],[4],[5],[6] We are reporting this as an unusual case where pelvic and abdominal sites were the locations of multiple primary extrauterine ESS and where there was a site of endometriosis contiguous with the tumor in the right parametrium, clearly indicating the origin of the tumor.

   Case report Top

A 42-year-old female presented at another hospital having noticed a mass on the anterior abdominal wall for about a month. She had borne two children and had two other pregnancies, which had ended in abortion. Ultrasonography of the abdomen at that hospital was reported as showing a cystic mass of 11 mm × 11 mm size with thick hyperechoic walls and echogenic mural nodules, located in the midline of the anterior abdominal wall. Ultrasonography then also reportedly showed an enlarged uterus and 8 mm thick endometrium. Both ovaries were normal in size and echogenicity.

A computed tomography scan of the brain and chest X-ray were done at the same admission and were reported as normal.

The cyst on the anterior abdominal wall was excised at that hospital, and the specimen blocks were sent to this laboratory for an opinion. In this hospital, we concurred with the diagnosis of ESS made earlier. The patient was referred here for further treatment.

At this hospital, a magnetic resonance imaging scan of the abdomen demonstrated multiple well-defined enhancing rounded nodular lesions in the pelvic cavity and pouch of Douglas. The uterus was bulky and free of tumor. The cervix and vagina appeared normal.

The patient underwent a total abdominal hysterectomy along with removal of all the pelvic and abdominal lesions and resection of pelvic and para-aortic lymph nodes.

The specimens received in this laboratory consisted of the uterus with the adnexae. On inspection there were multiple nodular firm white masses ranging in size from 0.7 cm to 2.8 cm in diameter [Figure 1] which were present in both parametria. When sectioned the nodules had homogeneous white and soft surfaces.
Figure 1: Gross picture showing nodules of tumor in left parametrium (thin arrow) and in posterior vaginal wall (thick arrow)

Click here to view

Similar nodules from the omentum, sigmoid mesentery and transverse mesocolon were also sent separate from the above. There were no intraparenchymal tumors in both ovaries, uterus and cervix on gross examination. The myometrium was thick and measured 2.5 cm.

On histological examination, the nodules were found to be composed of short fascicles of spindle-shaped cells with uniform small round to oval nuclei and rare mitoses [Figure 2]a. Small prominent arterioles were scattered among the stromal cells [Figure 2]b. A focus of endometriosis was intimately associated with the tumor in the right parametrium [Figure 2]a. The tumor cells stained positively on immunohistochemistry for CD10 [Figure 2]c and progesterone receptor [Figure 2]d, confirming the morphological diagnosis. The staining was diffuse and strong.
Figure 2: Composite photomicrograph showing low power field of endometriotic focus being contiguous with tumor (a); high power field of tumor with short fascicles of spindle-shaped cells (b); high power fields of positive immunostaining of tumor for CD10 (c) and progesterone receptor (d)

Click here to view

The right ovary was found to have a microscopic focus of tumor. The original biopsy site on the anterior abdominal wall had a residual focus of tumor. Multiple random sections of the uterine wall on histopathology revealed extensive adenomyosis, but no evidence of ESS. Metastatic deposits of ESS were present in two of the seven para-aortic lymph nodes.

On the 8 th postoperative day, the patient developed a rupture at the rectosigmoid junction from where adherent tumor was previously removed. This site was surgically repaired, and the patient recovered and was discharged on the 15 th day after surgery.

   Discussion Top

This is an unusual case of ESS. It spared the uterine wall but arose at various sites in the pelvic and abdominal peritoneum. One tumor in the right parametrium clearly demonstrated its origin from endometriosis.

It is estimated that only 0.7-1% of women with endometrosis develop malignant neoplasms and the most frequently involved transformation site is the ovary. [7] Most malignant tumors, which arise from endometriosis are usually endometrial adenocarcinomas. The development of ESS is rare. [4] In the case series of Yantiss et al. on gastrointestinal endometriosis, only one case of ESS occurred among the 17 cases studied. [2] Subsequently, other case reports of gastrointestinal ESS complicating endometriosis have appeared. [3],[4],[5],[6]

Two cases in literature were correctly diagnosed several years after hysterectomy in postmenopausal women, both of whom had a past history of endometriosis. [3],[6] One patient had an Estraderm patch as estrogen replacement therapy and it was speculated that this may be the cause of development of ESS in the postmenopausal state. [2],[5]

The largest series of extrauterine ESS reported in recent literature is from the M D Anderson Cancer Center, Texas from where 63 cases were collected over a 21-year period. [1] Most of the 63 cases were located in the abdominal peritoneum and were present at multiple sites, as seen in this case. The study showed that half the cases of ESS had demonstrable endometriosis to corroborate the diagnosis.

Endometrial stromal sarcoma can be a diagnostic challenge when encountered in the abdominal peritoneum. ESS should enter the differential diagnoses along with common spindle cell tumors of the abdominal peritoneum such as GIST and leiomyosarcoma. Immunocytochemistry plays an important role in distinguishing these tumors.

Complete surgical removal of ESS is the treatment of choice, as has been performed in this case. However, the metastasis of ESS to para-aortic lymph nodes, would usually predict an unfavorable outcome. Treatment of metastatic ESS with adjuvant hormonal therapy has been shown to be effective. [6]

   Conclusion Top

This case is being presented to emphasize the rarity of primary extrauterine ESS occurring in pelvic and abdominal soft tissue and to corroborate its association with endometriosis. ESS should always be part of the differential diagnoses of any pelvic/abdominal spindle cell tumor.

   References Top

Masand R, Euscher E, Deavers M, Malpica A. Extrauterine endometrial stromal sarcoma: A pathologic study of 63 cases with clinical correlation. Am J Surg Pathol 2013 Nov;37:1635-47.   Back to cited text no. 1
Yantiss RK, Clement PB, Young RH. Neoplastic and pre-neoplastic changes in gastrointestinal endometriosis: A study of 17 cases. Am J Surg Pathol 2000;24:513-24.  Back to cited text no. 2
Ayuso A, Fadare O, Khabele D. A case of extrauterine endometrial stromal sarcoma in the colon diagnosed three decades after hysterectomy for benign disease. Case Rep Obstet Gynecol 2013;2013:202458.  Back to cited text no. 3
Cho HY, Kim MK, Cho SJ, Bae JW, Kim I. Endometrial stromal sarcoma of the sigmoid colon arising in endometriosis: A case report with a review of literatures. J Korean Med Sci 2002;17:412-4.  Back to cited text no. 4
Mourra N, Tiret E, Parc Y, de Saint-Maur P, Parc R, Flejou JF. Endometrial stromal sarcoma of the rectosigmoid colon arising in extragonadal endometriosis and revealed by portal vein thrombosis. Arch Pathol Lab Med 2001;125:1088-90.  Back to cited text no. 5
Zemlyak A, Hwang S, Chalas E, Pameijer CR. Primary extrauterine endometrial stromal cell sarcoma: A case and review. J Gastrointest Cancer 2008;39:104-6.  Back to cited text no. 6
Soo-Kyung P, Sun JL. Endometrial stromal sarcoma associated with extrauterine endometriosis: A case report and literature review. Korean J Gynecol Oncol 2008;19:87-91.  Back to cited text no. 7

Correspondence Address:
Tanushree Ghosal
MD, DNB, Fellow, Oncopathology, LVM-14A, ABL Township, Durgapur - 713 206, West Bengal
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.138757

Rights and Permissions


  [Figure 1], [Figure 2]

This article has been cited by
1 Extrauterine endometrial stromal sarcoma: A systematic review and outcome analysis
Pratik Q. Deb, Debra S. Heller
Annals of Diagnostic Pathology. 2022; : 151966
[Pubmed] | [DOI]
2 Primary low-grade extrauterine endometrial stromal sarcoma with sex cord elements involving the omentum: A case report with review of literature
ChikkanagannaP Manjula, VSuguna Beluru, B.K. Hanumantha Raju, NainaMary Simon
Archives of Medicine and Health Sciences. 2022; 10(1): 87
[Pubmed] | [DOI]
3 Extragenital endometrial stromal sarcoma of transverse mesocolon: A diagnostic conundrum
Aishwarya Sharma, Munita Bal, Santosh Menon
International Journal of Surgical Pathology. 2021; : 1066896921
[Pubmed] | [DOI]
4 Disseminated Peritoneal Endometriosis Evolving to High-Grade Endometrial Stromal Sarcoma: A Case Report with Review of the Literature
Shruti Gupta, Parikshaa Gupta, Radhika Srinivasan, Subhash Chandra Saha, Seema Chopra, Pooja Sikka, Arvind Rajwanshi
Journal of Gynecologic Surgery. 2020; 36(4): 209
[Pubmed] | [DOI]
Krishna Govindan Balachandran Nair, Syamini Chandrasekharapillai Leelamany
Journal of Evolution of Medical and Dental Sciences. 2019; 8(16): 1355
[Pubmed] | [DOI]
6 Extrauterine endometrial stromal sarcoma arising in a background of endometriosis following prior total abdominal hysterectomy
Jonathan Chapman,Blake Bartholomew,Sayf Al-Katib
Journal of Endometriosis and Pelvic Pain Disorders. 2018; 10(3): 133
[Pubmed] | [DOI]
7 Primary extragenital endometrial stromal sarcoma of the lung: first reported case and review of literature
Lara Alessandrini,Francesco Sopracordevole,Giulio Bertola,Simona Scalone,Martina Urbani,Gianmaria Miolo,Tiziana Perin,Fabrizio Italia,Vincenzo Canzonieri
Diagnostic Pathology. 2017; 12(1)
[Pubmed] | [DOI]
8 An unexpected diagnosis of primary omental endometrial stromal sarcoma in a patient with acute right abdominal pain: A case report and review of literature
Vered Buchholz,George Kiroff,Markus Trochsler,Harsh Kanhere
International Journal of Surgery Case Reports. 2017; 36: 8
[Pubmed] | [DOI]


    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Email Alert *
    Add to My List *
* Registration required (free)  

   Case report
    Article Figures

 Article Access Statistics
    PDF Downloaded138    
    Comments [Add]    
    Cited by others 8    

Recommend this journal