Low-grade Endometrial Stromal Sarcoma with Intracaval or Intracardiac Extension: A Retrospective Study of 8 Cases

Purpose: This study aimed to improve the knowledge of low-grade endometrial stromal sarcoma (LG-ESS) with intracaval or intracardiac extension and tried to identify the potential risk factors and optimal treatment method influencing prognosis.Methods: We performed a retrospective review of eight LG-ESS patients with intracaval or intracardiac extension who underwent treatment at Peking Union Medical College Hospital between 2012 and 2020.Results: The median age at diagnosis was 44 years, ranging from 28 to 56 years. Abnormal uterine bleeding was the most common intimal symptom (3/8), followed by low back discomfort (2/8), edema of the lower limbs (2/8), abdominal pain (1/8), and dyspnea (1/8). All patients underwent resection of the intravascular and extravascular portions of the tumor. Two patients were in stage IIIC, and 6 were in stage IVB. After surgery, 4 patients received adjuvant radiotherapy, of whom 3 also received letrozole. One patient was treated with letrozole alone, and 1 patient received medroxyprogesterone. The average follow-up time was 34.5 months, ranging from 6 to 98 months. No patients died or relapsed during the follow-up period.Conclusions: LG-ESS with intracaval or intracardiac extension is an uncommon type of tumor which is easily misdiagnosed and can only be diagnosed by histological evaluation after surgery. Complete tumoral excision followed by adjuvant therapy may benefit patient survival time. Long-term follow-up is essential due to the high rate of late recurrence.

Highly Symptomatic Progressing Cardiac Paraganglioma With Intracardiac Extension Treated With 177Lu-DOTATATE: A Case Report

IntroductionPrimary cardiac paragangliomas are rare tumors. Metastatic disease is even rarer. Surgical management is technically challenging, and sometimes even impossible. Available therapeutic modalities for metastatic disease include external beam radiation therapy as well as systemic treatments, namely 131I-MIBG and more recently, peptide receptor radionuclide therapy (PRRT) with 177Lu-DOTATATE. To our knowledge, this is the first case of progressive unresectable cardiac paraganglioma with intracardiac extension treated with dosimetry based personalized PRRT to be reported. This case is of particular interest since it documents for the first time the efficacy, and especially the safety of the 177Lu-DOTATATE PRRT in this precarious context for which therapeutic options are limited.Case PresentationA 47-year-old man with no medical history consulted for rapidly decreasing exercise tolerance. The investigation demonstrated an unresectable progressing metastatic cardiac paraganglioma with intracardiac extension. The patient was treated with personalized 177Lu-DOTATATE PRRT and showed complete symptomatic and partial anatomical responses, with a progression-free survival of 13 months.ConclusionsPRRT with 177Lu-DOTATATE should be considered for inoperable cardiac paraganglioma. No major hemodynamic complications were experienced. Therapy resulted in safety and substantially improved quality of life.

Renal angiomyolipoma with inferior vena cava and right atrium extension in patient with tuberous sclerosis complex: a rare case report and literature review.

Angiomyolipoma(AML) is one of the most common benign renal tumors. Classical AML is benign, but it can be locally invasive, extending into perirenal fat, or in rare cases, invading the renal collecting system, renal vein or inferior vena cava and right atrium. About 10% of patients clinically diagnosed with renal AML have tuberous sclerosis complex. Tuberous sclerosis complex can be diagnosed by genetic diagnosis or clinical manifestation. We report a rare case of a 35-year-old woman who was diagnosed with tuberous sclerosis complex caused by TSC2 gene mutation, which was characterized by multiple angiomyolipoma in the right kidney and extended growth to the inferior vena cava and right atrium. Intracardiac extension is often observed in the malignant tumor and only seldom seen in benign tumors. Our case reminds the rare possibility of intracardiac extension in renal AML, which may potentially result in fatal complications if not appropriately managed.

Intracardiac extension of chondroblastic osteosarcoma

Intravascular tumour extension invading the intracardiac space is rarely seen with osteosarcoma. We present a patient with a history of previously resected pelvic osteosarcoma who was later found to have a local recurrence with continuous intravascular extension from the right femoral vein to the right atrium. Preoperative imaging studies initially described extensive thrombus burden, and a multidisciplinary approach involving open and percutaneous thrombectomy was planned. Intraoperative inspection and pathological analysis revealed unresectable malignant solid tumour rather than thrombus. Though exceedingly rare, the possibility of metastatic tumour must be considered when planning treatment strategies for these patients.

Intravenous Leiomyoma with Intracardiac Extension: A Rare Late Presentation After Hysterectomy

Intravenous leiomyoma is a rare, benign smooth muscle tumor arising from intrauterine venules or the myometrium. It can rarely present as intracardiac mass long after hysterectomy. In this case report we describe a 40-year-old female with previous history of hysterectomy, presenting with an intracardiac mass which was successfully managed with single stage tumor resection under cardiopulmonary bypass. Subsequent histopathology showed features of leiomyoma. The diagnosis of intravenous leiomyoma with cardiac extension should be kept in a female patient presenting with intracardiac mass with previous history of myomectomy or hysterectomy.