Search

Splenic stromal sarcomas are rarely reported tumours that were previously grouped as non-angiomatous, non-lymphomatous mesenchymal neoplasms of the canine spleen. Highly variable survival times have been reported probably due to their heterogeneous nature. The purpose of this study was to assess the outcome and prognostic factors in dogs with splenic stromal sarcoma after treatment by splenectomy. Clinical data were collected retrospectively and histopathology was reviewed for 47 patients. Histological classification, based on morphology in haematoxylin and eosin (HE)-stained sections, in conjunction with immunolabelling of macrophage scavenger receptor-A (CD204), desmin, factor VIII-related antigen and smooth muscle actin (SMA), yielded diagnoses of undifferentiated stromal sarcoma (n = 22), complex nodular hyperplasia (CNH, n = 9), sarcoma arising from benign complex nodular hyperplasia (n = 3), histiocytic sarcoma (n = 3), haemangiosarcoma (n = 1) and leiomyosarcoma (n = 1). Four samples were excluded from analysis due to extensive necrosis. An anti-podoplanin (PDPN) antibody was validated on canine tissue and used to assess expression of this protein as a potential indicator of the tissue of origin of the neoplasms (28/42 tumours were positive). There was a statistically significant difference in survival time between patients with stromal sarcoma (sarcoma from benign CNH and undifferentiated stromal sarcoma) and CNH (178 d versus 637 d, respectively; P = 0.027). Dogs with stromal sarcomas and high mitotic count (≥9 per 10 high-power fields) had a significantly shorter survival time (67 d versus 439 d; P = 0.01). Clinical diagnosis of splenic tumours should include evaluation for the presence of benign nodular hyperplasia morphology and immunohistochemistry to exclude more aggressive malignancies where adjuvant therapy is recommended. As in humans, PDPN may be an effective marker for stromal sarcomas of the canine spleen and immunopositivity suggests a fibroblastic reticular or follicular dendritic cell origin.

Endometrial stromal sarcoma (ESS) is a rare malignant tumor that constitutes about 0.2% of all uterine malignancies and 10% of uterine sarcomas. ESS is generally misdiagnosed as leiomyoma or endometrial polyp and typically discovered on histopathological examination postoperatively because of its rarity. Endometrial stromal tumors are composed of cells resembling normal endometrial stroma in its proliferative phase. The histologic diagnosis of the high grade is made if there is a high-grade sarcoma with a high mitotic index and nuclear anaplasia. The mean age of presentation of high-grade endometrial sarcoma is about 61 years with the most common presenting complaint is menorrhagia. The median overall survival for high-grade endometrial sarcoma is 53 months with optimal cytoreduction. A 49-year-old woman P2 L2 presented with nonspecific complaint of discharge and spotting per vaginum. In the present case, the provisional diagnosis by clinical findings as well as imaging was in favor of the inversion of submucous fibroid. Preoperative histopathological examination and immunohistochemistry confirmed the diagnosis of high-grade undifferentiated ESS. Haultain’s operation followed by total abdominal hysterectomy and bilateral salpingo-oophorectomy was performed. The patient was referred to another center for radiotherapy. From there, she was lost to follow-up. Rarity of endometrial stromal tumor limits the clinician view to diagnose it preoperatively. We were fortunate to have preoperative histopathological diagnosis of ESS. Furthermore, as ESS is rare and undifferentiated stromal sarcoma is even rarer, literature is lacking on its optimal management. Hence, it is important for all clinicians to keep the high degree of suspicion for ESS while working up any case of abnormal uterine bleeding.

Beyond leiomyosarcoma, uterine sarcomas and tumors that contain “sarcoma” in the name (i.e., carcinosarcoma) are well-recognized biological entities. The three nonleiomyosarcoma tumors, (low-grade) endometrial stromal sarcoma, high-grade undifferentiated stromal sarcoma, and mixed Mullerian tumors including carcinosarcoma, are all very different from one another biologically. They are often omitted in discussions of soft tissue pathology as different groups of pathologists generally review such cases in expert centers than those who review soft tissue or bone tumors. Age distribution for adult uterine endometrial stromal tumors is shown in Fig. 21.1.

Only 0.2-1% of all mammary malignancies are sarcomas of the breast. This study includes 4 cases: 2 osteosarcomas, 1 fibrosarcoma, and 1 malignant undifferentiated stromal sarcoma. The therapy was mastectomy in 3 cases with dissection of axillary lymph nodes and simple mastectomy in one case. One patient demonstrated local recurrence and died. The remaining 3 patients developed neither metastases nor local recurrence. They are still alive after a follow-up period of between 18 months and 17 years. As first-line treatment, wide local excision or simple mastectomy is recommended. Dissection of the axillary lymphatics, adjuvant radiotherapy, or chemotherapy have no established value in the treatment of breast sarcoma. In our 4 cases, flow-cytometric analysis does not always agree with the biological properties of the tumour and the clinical behaviour, in contrast to the results obtained in respect of carcinomas.