INTRODUCTION: Low-grade endometrial stromal sarcoma (LGESS) is an uncommon uterine neoplasm, which has a highly recurrent nature. We report a case of LGESS which presented with vaginal bleeding and incidental chest radiograph findings of multiple pulmonary nodules which on work up revealed as pulmonary metastasis of LGESS.
CASE PRESENTATION: 43 years old Caucasian female presented to her primary care physician for 6 months history of Menometrorrhagia,continuous vaginal bleeding for past 25 days, Dyspnea on exertion, excessive fatigue for 2 months and lower abdominal pain with vague lump for 3–4 months. Her past medical history was significant for DUB. She was receiving no medications. She has 30 pack year smoking history. Positive physical findings limited to significant pallor, Non tender palpable mass palpable in the hypogastric area up to the umbilicus. Significant Laboratory findings include Iron deficiency Anemia (Hemoglobin-5.4g/dl), Chest radiograph(Fig-1) showed multiple, non-calcified, variable sized, round-shaped nodules throughout both lungs. Patient was admitted to the hospital for blood transfusion due to anemia. CT Scan of Chest (Fig-2) showed bilateral numerous noncalcified nodules in lungs. The largest nodule in the left lung is about 1.5 cm. Uterus remarkably enlarged measuring 12.4 cm X 10.7 cm. Dilation and curettage was done which was negative for malignancy. PET scan was performed for the same reason and reported as, markedly enlarged uterus with a moderately hypermetabolic endometrial mass. Multiple pulmonary nodules had no significant FDG uptake to suggest an FDG-avid malignancy. Cardiothoracic surgery was consulted. Patient underwent Video Assisted Thoracoscopic Surgery with wedge biopsy of the lung nodules(Fig-3) showing metastatic LGESS. The tumor cells stained uniformly for CD10, estrogen receptor (ER), progesterone receptor (PR) and vimentin. Patient then underwent Total Abdominal Hysterectomy with Bilateral Salpingo Oophorectomy. Lower uterine segment was involved by the tumor. Histopathology showed endometrial stromal cells with no mitotic activity (characteristic feature of low grade). Neoplastic cells are focally disposed in a whorled pattern around numerous arterioles. Tumor growth was observed in the myometrial veins and in lymphatics. Diagnosis of LGESS was confirmed and tumor was staged as IV-B LGESS. Patient was started on hormonal therapy (progestin) after TAH.
DISCUSSIONS: The etiology of multiple pulmonary nodules is broad, with metastatic disease being the most common. Metastatic ESS should be included in the differential diagnosis of nonepithelial neoplasms in women. ESS represents 0.2% of all uterine malignancies and up to 25% of primary uterine sarcomas. Histologically, they can be divided into High & Low grades based on their mitotic count (more or less than 10 mitoses per 10 high power fields, respectively). The microscopic appearance of LGESS is characteristic with sheets of densely packed, uniform, small spindle or ovoid cells, often with prominent hyalinization within stromal vessels. The tumor also has a tendency to extend into blood vessels and lymphatic's and may be expressed as worm like masses(1). Lung metastases occur commonly and have been noted at the initial diagnosis of ESS as well as many years even after the removal of the primary. LGESS has a relatively indolent clinical course with a 5-year survival of approximately 60%. Favorable prognostic characteristics include early tumor stage, low myometrial invasion and low mitotic rate. Treatment options include hysterectomy for removal of primary and hormonal therapy for metastasis. Progestins have been the most widely used agents aiming at the inhibition of estrogen-induced growth promotion(2).
CONCLUSION: This case report is a perfect example of common clinical presentation of a rare and indolent tumour, LGESS which often metastasis to the lungs. Due to the rarity of the presentation, the condition is often missed or diagnosed late.
DISCLOSURE: Srikanth Davuluri, None.