Endometrial stromal sarcomas of uterus are quite rare. Most of the recurrences in these tumors are seen in the pelvis. However, extrapelvic recurrences and metastases to other parts are quite unusual. Here, we are reporting a rare case of caecal recurrence of endometrial stromal sarcoma.
Endometrial stromal sarcomas (ESS) are a rare variant of malignant mesenchymal tumors. This disease entity belongs to the rarest uterine malignancies (prevalence category < 1–9/1,000,000) [
A 52-year-old female was subjected to total abdominal hysterectomy with bilateral salpingo-oophorectomy (TAHBSO) for LG-ESS. Patient received radiotherapy and was doing well for 10 years. After 10 years, she presented with the right lower abdominal discomfort and a lump. USG showed an ill-defined mass with heterogenous echogenicity in the right lower quadrant of the abdomen. MRI revealed a well-defined regular contour lesion, measuring 5.3 × 4.8 cm with isointense signals on T2-weighted images, anteromedial to right external iliac vessels (Figure
Well-defined lesion, anteromedial to the right external iliac vessels.
PET/CT scan (Figure
PET/CT scan showing well-defined heterogeneously enhancing lesion in the right iliac fossa along the anterior abdominal wall.
Tumor markers (Ca125 and CEA) and other baseline investigations were within normal limits. Operative findings revealed 5 × 4 cm solid thin pedicled, well encapsulated, and mobile mass arising from the caecum, without being adherent to surrounding bowel loops or other structures (Figure
Intraoperative picture showing pedunculated mass arising from the caecum.
Histopathological examination revealed features of high-grade endometrial stromal sarcoma (Figure
Hematoxylin and eosin stain of caecal endometrial stromal sarcoma, showing round to oval spindle-shaped nuclei with nuclear atypia.
Immunohistochemistry picture showing CD10 positivity.
Immunohistochemistry picture showing estrogen receptor positivity.
Immunohistochemistry picture showing progesterone receptor positivity.
Low-grade endometrial stromal sarcomas (LG-ESS) are usually slow-growing neoplasms with an indolent clinical course, whereas high-grade ESS often show more aggressive nature. For LG-ESS, total abdominal hysterectomy is recommended as primary treatment, whereas the need for bilateral salpingo-oophorectomy, adjuvant chemotherapy, radiotherapy, or hormonal therapy is still controversial [
Recurrence develops in one-third to one-half of patients with LG-ESS. It has been reported even 30 years after the initial treatment [
Li and Chang [
ESS may arise as a primary extrauterine endometrial sarcoma (EESS), mainly on the background of endometriosis, with predominant gonadal involvement. Very rarely does EESS arise primarily in the gastrointestinal or extragastrointestinal tract organs outside the pelvis or evolve without preceding endometriosis [
The main differential diagnosis for EESS when arising from bowel is some other mesenchymal tumor, especially gastrointestinal stromal tumor (GIST). Spindle cells in EESS are characterized by monotonous arrangement, compared with sheets or fascicle arrangement in GIST. Proliferation of small arterioles, resembling spiral arterioles of the endometrium, is also typical of EESS. Mucocele of appendix can also present in this manner. However, the final diagnosis, especially in cases of unusual tumor location and absence of endometriosis, depends on immunohistochemistry. Positive labelling for CD10, PR, and ER with no reactivity for CD117 and CD34 confirms the diagnosis of ESS [
Recurrence of endometrial stromal sarcoma arising in the caecum is rare. Postoperative hormone therapy is important in such cases. Besides, this entity even though rare should be kept in mind for differential diagnosis of a caecal mass.
The authors declare that there is no conflict of interest regarding the publication of this paper.
The authors acknowledge the Department of Pathology, SKIMS, for providing photomicrographs of the case.