Rare Pathology Case Report: Low-Grade Endometrial Stromal Sarcoma Forming Sex Cord– and Endometrioid Gland–Like Differentiation in Metastatic Foci
Low-grade endometrial stromal sarcomas (LGESSs) are indolent tumors with a slow progression rate that tend to recur locally. They represent up to 10% of all primary sarcomas of the uterus and endometrium and only 0.2% of all genital tract tumors. They are commonly present in a younger demographic co...
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Wiley
2024-01-01
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| Series: | Case Reports in Pathology |
| Online Access: | http://dx.doi.org/10.1155/2024/4073869 |
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| author | Haneen Al-Maghrabi Ghadeer Mokhtar Jaudah Al-Maghrabi |
| author_facet | Haneen Al-Maghrabi Ghadeer Mokhtar Jaudah Al-Maghrabi |
| author_sort | Haneen Al-Maghrabi |
| collection | DOAJ |
| description | Low-grade endometrial stromal sarcomas (LGESSs) are indolent tumors with a slow progression rate that tend to recur locally. They represent up to 10% of all primary sarcomas of the uterus and endometrium and only 0.2% of all genital tract tumors. They are commonly present in a younger demographic compared to other uterine tumors, with patients’ ages typically between 42 and 58 years old. Although the overall 5-year survival rate is excellent, it has a natural history of delayed metastases which may manifest even decades after the disease was first diagnosed. They typically present as poorly defined lesions infiltrating the myometrium, along with extensive engagement of surrounding vascular structures. LGESS may display variants of different morphologies such as smooth muscle, fibromyxoid, sex cord-like, and endometrioid-type gland differentiation. These variations can pose a diagnostic challenge. The occurrence of this differentiation in a metastatic focus rather than in the primary tumor is seldom recorded in the literature. We present a case of a 51-year-old lady with a history of LGESS who was treated with surgery and radiotherapy and then presented after 12 years with an inferior vena cava (IVC) mass, which was confirmed histologically to be metastatic LGESS. Immunohistochemistry studies reveal strong positivity for CD10, WT1, and PR. These markers were negative in the sex cord and endometrioid gland–like differentiation counterparts. The patient had her initial follow-up appointment after the IVC mass resection, and she was in good health with no complications. To the best of our knowledge, this case represents a unique instance of metastatic LGESS exhibiting both sex cord and endometrioid gland–like differentiation that has not been observed in the primary tumor. |
| format | Article |
| id | doaj-art-5df22814b2034a199b2e80902f4d94c7 |
| institution | Kabale University |
| issn | 2090-679X |
| language | English |
| publishDate | 2024-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Pathology |
| spelling | doaj-art-5df22814b2034a199b2e80902f4d94c72025-02-03T10:24:54ZengWileyCase Reports in Pathology2090-679X2024-01-01202410.1155/2024/4073869Rare Pathology Case Report: Low-Grade Endometrial Stromal Sarcoma Forming Sex Cord– and Endometrioid Gland–Like Differentiation in Metastatic FociHaneen Al-Maghrabi0Ghadeer Mokhtar1Jaudah Al-Maghrabi2Department of Pathology and Laboratory MedicineDepartment of Pathology and Laboratory MedicineDepartment of Pathology and Laboratory MedicineLow-grade endometrial stromal sarcomas (LGESSs) are indolent tumors with a slow progression rate that tend to recur locally. They represent up to 10% of all primary sarcomas of the uterus and endometrium and only 0.2% of all genital tract tumors. They are commonly present in a younger demographic compared to other uterine tumors, with patients’ ages typically between 42 and 58 years old. Although the overall 5-year survival rate is excellent, it has a natural history of delayed metastases which may manifest even decades after the disease was first diagnosed. They typically present as poorly defined lesions infiltrating the myometrium, along with extensive engagement of surrounding vascular structures. LGESS may display variants of different morphologies such as smooth muscle, fibromyxoid, sex cord-like, and endometrioid-type gland differentiation. These variations can pose a diagnostic challenge. The occurrence of this differentiation in a metastatic focus rather than in the primary tumor is seldom recorded in the literature. We present a case of a 51-year-old lady with a history of LGESS who was treated with surgery and radiotherapy and then presented after 12 years with an inferior vena cava (IVC) mass, which was confirmed histologically to be metastatic LGESS. Immunohistochemistry studies reveal strong positivity for CD10, WT1, and PR. These markers were negative in the sex cord and endometrioid gland–like differentiation counterparts. The patient had her initial follow-up appointment after the IVC mass resection, and she was in good health with no complications. To the best of our knowledge, this case represents a unique instance of metastatic LGESS exhibiting both sex cord and endometrioid gland–like differentiation that has not been observed in the primary tumor.http://dx.doi.org/10.1155/2024/4073869 |
| spellingShingle | Haneen Al-Maghrabi Ghadeer Mokhtar Jaudah Al-Maghrabi Rare Pathology Case Report: Low-Grade Endometrial Stromal Sarcoma Forming Sex Cord– and Endometrioid Gland–Like Differentiation in Metastatic Foci Case Reports in Pathology |
| title | Rare Pathology Case Report: Low-Grade Endometrial Stromal Sarcoma Forming Sex Cord– and Endometrioid Gland–Like Differentiation in Metastatic Foci |
| title_full | Rare Pathology Case Report: Low-Grade Endometrial Stromal Sarcoma Forming Sex Cord– and Endometrioid Gland–Like Differentiation in Metastatic Foci |
| title_fullStr | Rare Pathology Case Report: Low-Grade Endometrial Stromal Sarcoma Forming Sex Cord– and Endometrioid Gland–Like Differentiation in Metastatic Foci |
| title_full_unstemmed | Rare Pathology Case Report: Low-Grade Endometrial Stromal Sarcoma Forming Sex Cord– and Endometrioid Gland–Like Differentiation in Metastatic Foci |
| title_short | Rare Pathology Case Report: Low-Grade Endometrial Stromal Sarcoma Forming Sex Cord– and Endometrioid Gland–Like Differentiation in Metastatic Foci |
| title_sort | rare pathology case report low grade endometrial stromal sarcoma forming sex cord and endometrioid gland like differentiation in metastatic foci |
| url | http://dx.doi.org/10.1155/2024/4073869 |
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