Pulmonary artery mass with PIK3CA mutation after orthotopic heart transplantation.

BACKGROUND: Patients can develop de novo malignancies following orthotopic heart transplantation. However, vascular tumors are not commonly described in this population. CASE PRESENTATION: We present a 69-year-old female with a history of orthotopic heart transplantation for chemotherapy-induced cardiomyopathy who developed an incidental pulmonary artery mass six years after her transplantation. Given concerns for malignancy, the patient underwent an operative excisional biopsy through a left anterior mini-thoracotomy with femoral artery and vein cannulation for cardiopulmonary bypass. The mass was determined to be a non-malignant vascular overgrowth with PIK3CA mutation. CONCLUSION: We present the case of an unusual pulmonary artery mass with PIK3CA mutation found in a post heart transplant patient. We were able to spare her the morbidity of a redo-sternotomy by excising the mass via a minimally invasive left anterior thoracotomy approach.

Extravascular lipoma over the subclavian vein.

A rare instance of extravascular lipomas located over the subclavian vein in a woman in her early 60s presented unique diagnostic and therapeutic challenges due to their close proximity to critical structures. Lipomas, which are the most commonly occurring benign soft tissue tumours, are typically composed of mature adipocytes. Although lipomas can develop in any region of the body, they are most commonly found in the subcutaneous tissues of the trunk and proximal limbs. Extravascular lipomas, especially those near major blood vessels like the subclavian vein, are extremely uncommon.In this particular case, the tumour was extensively in contact with the right subclavian vein, though there was no intravascular involvement. The lipomatous tumour was successfully excised during surgery, with the subclavian vein and surrounding structures being preserved. Histopathological analysis confirmed the diagnosis of a benign lipoma. This case highlights the significance of early diagnosis, meticulous surgical planning and multidisciplinary collaboration to achieve the best possible outcomes.

Single-stage resection of uterine fibroids and intravascular leiomyomatosis: a case report.

BACKGROUND: Uterine Fibroids (UFs) are common benign tumors in the female reproductive tract, but their progression to intravascular leiomyomatosis (IVL) is rare. Presently, there are few reports on single-stage resection of UFs and IVL. CASE PRESENTATION: A 47-year-old woman, G2P2, had been diagnosed multiple UFs four years ago and now developed heart failure. Imaging examinations revealed that UFs had invaded the right iliac vein and extended into the right atrium through the inferior vena cava. Through multidisciplinary collaboration and a single-stage resection, the patient has survived for over 24 months post-surgery, and her heart function has significantly improved compared to preoperative levels, with no recurrence of UFs observed. CONCLUSIONS: Single-stage resection of IVL and UF is feasible and advantageous for this case, and selecting the appropriate surgical approach is crucial.

Intravenous leiomyomatosis in the inferior vena cava and right atrium with pulmonary benign metastasizing leiomyoma secondary to a pelvic arteriovenous fistula: A case report and literature review.

BACKGROUND: To report the diagnosis and treatment of a rare disease of intravenous leiomyomatosis (IVL) originating from the uterus, growing in the inferior vena cava (IVC) and extending into the right atrium (RA) associated with a pelvic arteriovenous fistula (AVF). This is the first reported case of IVL in the IVC and RA with pulmonary benign metastasizing leiomyoma (PBML) secondary to a pelvic AVF despite the use of GnRH agonists in a nonmenopausal woman. CASE PRESENTATION: The patient was a 50-year-old premenopausal woman with a history of surgical resection for and antiestrogen conservative drug for pulmonary benign metastasizing leiomyoma (PBML) 5 years. The patient nevertheless developed IVL in the IVC, internal iliac vein and RA accompanied by AVF. Vaginal ultrasound combined with echocardiography and computerized tomographic venography imaging assists in the diagnosis of IVL combined with AVF, with histopathology and immunohistochemistry ultimately confirming the diagnosis. The patient ultimately was performed with a combination of hysterectomy, bilateral adnexectomy, and resection of tumors in the IVC and RA without cardiopulmonary bypass and sternotomy. CONCLUSION: BML may be difficult to control with incomplete removal of the uterus and ovaries even with the use of antiestrogenic medications, and medically induced AVF resulting from fibroid surgery may accelerate this process and the development of IVL.

Resecting Lower Segment Inferior Vena Cava Leiomyosarcoma With Middle Segment Extension While Avoiding Renal Morbidity.

Primary leiomyosarcoma of the inferior vena cava (IVC) is a rare and aggressive mesenchymal tumor, with less than 400 reported cases to date. Complete resection of the tumor with clear margins is the only proven curative treatment, providing survival benefits. Nonetheless, leiomyosarcomas in the middle segment or those extending up to it within the inferior vena cava (IVC) frequently necessitate renal reimplantation or nephrectomy, with rates varying between 56% and 75%. In this case report, we present a 65-year-old female with lower segment IVC leiomyosarcoma with middle segment extension, successfully resected and reconstructed while avoiding associated renal reimplantation or nephrectomy morbidity.

Vena cava leiomyosarcoma surgery results in a retrospective cohort of 41 patients from two centers.

BACKGROUND: Leiomyosarcoma of the vena cava (LMS-VC) is a rare entity with poor oncological outcomes and a lack of histological staging prognostic factors. METHODS: Outcomes of consecutive patients operated on LMS-VC between March 2003 and May 2022, in two specialized sarcoma centers were reported. RESULT: Forty-one patients were identified. Median size of LMS-VC was 9 cm with 68% of complete obstruction. After surgery, severe complication rate was 30%. No postoperative mortality was reported. Microscopic complete excision was obtained for 71% of patients, R1 for 27% and one patient presented an R2 resection. Grade 3 was found in 24%. After a median follow-up of 70 months, 3 years disease-free survival (DFS) and 5 years DFS were 34% and 17%, and 3 years overall survival (OS) and 5 years OS were 74% and 50%. Distant metastasis concerned 54% of recurrences, local 7% and local and distant 5%. Multivariate analysis showed that FNCLCC grade (p < 0.001) and perioperative chemotherapy (p = 0.026) were significant factors for DFS. In multivariate analysis, FNCLCC grade was a significant factor for OS (p = 0.004). DISCUSSION: Perioperative chemotherapy may have a role to play in lowering the risk of recurrence for LMS-VC, particularly in high-grade tumor.

Ex Situ Liver Resection and Autotransplantation with Retrohepatic Inferior Vena Cava Reconstruction and Atrial Thrombectomy Under Extracorporeal Circulation for Inferior Vena Cava Leiomyosarcoma.

BACKGROUND: Surgery is the only curative treatment for retrohepatic inferior vena cava (r-IVC) leiomyosarcoma.1 Cavo-hepatic confluence invasion is a poor prognostic situation, requiring extreme liver surgery for selected patients to achieve R0 margins (a crucial prognostic factor). Ex situ liver resection and autotransplantation (ELRA), developed by Pichlmayr et al., permits to achieve such R0 margin.2,3 METHODS: An 84-year-old patient in excellent condition (ECOG 0), without relevant past medical history, was referred for abdominal mass, bilateral lower limbs edema, and dyspnea. Workup revealed a large r-IVC leiomyosarcoma invading cavo-hepatic confluence and protruding in right atrium without any metastasis. After multidisciplinary consultation, surgical treatment was retained. Preoperative transoesophaegal echocardiography confirmed a 4-cm protruding tumoral thrombus in right atrium without abdominalisation possibility. RESULTS: A sterno-laparotomy was performed, consisting of a right nephrectomy for exposure and en bloc total hepatectomy comprising r-IVC after atriotomy for intracardiac thrombectomy under extracorporeal circulation. Tumorectomy (rIVC + segment I and IX) was performed on back table followed by a r-IVC reconstruction through a tubulized homologous venous patch. Native IVC was reconstructed as well, permitting a side-to-side cavo-caval anastomosis for liver reimplantation. Postoperative evolution was eventless except for an early bile leak that required surgical exploration. The patient was discharged on postoperative day 32. Pathological examination confirmed r-IVC-leiomyosarcoma T4N0M0 R0, FNCLCC grade 2. Eight months after surgery, general status was conserved with disappearance of symptoms, and IVC was permeable without leiomyosarcoma recurrence. CONCLUSION: Ex situ liver resection and autotransplantation with atrial thrombectomy is a surgical possibility for R0 r-IVC leiomyosarcoma invading cavo-hepatic confluence in selected patients.

A rare case of epithelioid angiosarcoma.

Epithelioid angiosarcoma is a rare high-grade vascular neoplasm with a poor prognosis. We present an anticoagulated 77-year-old man, with a history of popliteal/soleal vein thrombosis in the previous month, complaining of ipsilateral persistent lower limb pain and claudication. Absent popliteal/distal pulses prompted an arterial doppler ultrasound (DUS), revealing thrombosis of the distal superficial femoral artery and a popliteal mass. As the arterial wall's integrity could not be appropriately evaluated by DUS, adventitial cystic disease of the popliteal artery was suspected. Computed tomography angiography and magnetic resonance imaging findings were also suggestive. Due to refractory pain, he was submitted to a popliteal mass excision along with a femoral-posterior tibial bypass. Pathology revealed an epithelioid angiosarcoma. He was referred to a Sarcoma Center, requiring hospitalization for agitation and fever. A positron emission tomography (PET) scan revealed extensive lower limb disease persistence and distant metastases. He died on the 56th day after surgery. To our knowledge, there are only 15 cases of angiosarcoma of the popliteal artery described in the literature. Ours stands out as the first one unrelated to a popliteal aneurysm. Being a highly-aggressive tumor, an early diagnosis is challenging but essential to a successful treatment, warranting the need for suspicion of this neoplasm. An early core biopsy or surgical sample may expedite the diagnosis.

Pulmonary artery sarcoma affecting the pulmonary valve mistaken as pulmonary vasculitis: a case report and comparative literature review.

Pulmonary arterial sarcomas (PAS) are rare aggressive tumours occurring mainly in the pulmonary trunk. We report a case of PAS involving the pulmonary trunk wall and valve, with uniform wall thickening which represents an atypical imaging manifestation of this tumour. A 63-year-old male presented with vague respiratory symptoms with rapid progression. CTPA showed low density filling defects in both pulmonary arteries and PET scan showed increased uptake in the pulmonary trunk, which along with raised ESR suggested Pulmonary Vasculitis. Echo imaging showed Right ventricular hypertrophy and pulmonary stenosis. Response to steroid therapy was minimal and his symptoms worsened. A referral for second opinion was made and he was diagnosed with PAS. He underwent Pulmonary thromboendarterectomy with Pulmonary valve replacement. Post-operative histopathology confirmed the diagnosis. PAS is rare and frequently misdiagnosed. Surgical resection is not curative, but together with chemotherapy can prolong survival.

En Bloc Inferior Vena Cava (IVC) Resection Without Reconstruction With Right Hepatectomy and Right Nephrectomy for a Large IVC Leiomyosarcoma.

BACKGROUND: Radical resection remains the only potential cure in the management of inferior vena cava (IVC) leiomyosarcomas with multivisceral resections often needed (Borghi et al. in J Cardiovasc Surg (Torino) 63:649-663, 2022). This video describes the technical nuances of surgical resection of a large retrohepatic IVC leiomyosarcoma. PATIENT AND METHODS: Computed tomography of a 60-year-old woman revealed a 12 × 12 × 9.5 cm mass in the right suprarenal region infiltrating the IVC with intraluminal extension up to the hepatic venous confluence. The mass involved the right hepatic vein with infiltration of segment 7 of the liver and splaying of the right portal vein. Robust lumbar venous drainage from the infratumoral IVC was seen. En bloc IVC resection without reconstruction along with a right hepatectomy and right nephrectomy was performed via a right thoracoabdominal approach. RESULTS: After a Catell-Braasch maneuver, the surgery can be broadly divided into four major steps: (1) Right retroperitoneal mobilization of the tumor and right kidney with infratumoral IVC control, (2) mobilization of the right liver with suprahepatic IVC control, (3) division of the right portal structures with right hepatectomy, and (4) en bloc resection of the IVC tumor. Reconstruction of the IVC was not performed owing to the presence of venous collaterals (Langenbecks et al. in Arch Surg 407:1209-1216, 2022). Final histopathology showed a high-grade leiomyosarcoma with histologic organ invasion in the liver and right kidney with resected margins free of the tumor (R0). CONCLUSIONS: Meticulous preoperative planning and expertise in liver resection and retroperitoneal surgeries facilitates such radical yet safe multivisceral resection for a large retrohepatic IVC leiomyosarcoma without the need for a cardiopulmonary bypass.

Surgical outcomes of patients with inferior vena cava leiomyosarcoma.

INTRODUCTION: Primary vascular leiomyosarcomas are incredibly rare and have a poor prognosis. The purpose of this study was to analyze the surgical outcomes of patients with primary inferior vena cava (IVC) leiomyosarcoma. METHODS: We performed a retrospective review of IVC leiomyosarcoma resections performed at a single tertiary care hospital from 2014 to 2023. A total of 13 cases were analyzed, including 10 women and 3 men. The presenting symptoms, tumor characteristics, operative management, postoperative complications, and survival rates were assessed for each patient. RESULTS: The median patient age was 59 years (quartile [Q]1, 52 years; Q3, 68 years). The median tumor size was 7.0 cm (Q1, 6 cm; Q3, 12 cm). The median mitotic rate was 6 per 10 high-power fields (Q1, 2.5; Q3, 15.5). All 13 patients underwent grossly negative tumor resection, with 9 (69%) having microscopically negative margins (R0). No patient had lymph node involvement. The IVCs were managed with ligation in four patients for tumors already occluding the IVC and bovine pericardial patch angioplasty in seven patients or primary repair in two patients for patent IVCs. Concomitant right nephrectomy was performed in seven patients. Left renal vein ligation was performed in three patients, but no left nephrectomies were performed. Significant postoperative complications included one patient with lower extremity compartment syndrome, two patients with severe leg swelling, and one patient with arm swelling. The 30-day mortality rate was zero. Using the Kaplan-Meier product limit method, disease-specific survival was estimated to be 93%. CONCLUSIONS: Surgical resection is a feasible and effective oncologic treatment option for patients with IVC leiomyosarcoma. The IVC can be safely managed by ligation, primary repair, or patch angioplasty, depending on the prior patency of the IVC.

Clinical outcomes after inferior vena cava resection for malignant disease. A single center experience of 51 vena cava resections.

BACKGROUND AND OBJECTIVE: For tumors involving inferior vena cava (IVC), surgery with complete resection remains the first line treatment. Management of IVC after resection, either ligation without reconstruction or primary reconstruction, is debated. Our study aimed to evaluate type of venous reconstruction, anticoagulation management and morbidity. METHODS: A French single center database of patients who underwent partial or total circumferencial resection of the IVC for malignant disease was analyzed. Inclusion criteria were any oncologic procedure for a retroperitoneal neoplasm requiring concomitant resection of the IVC with or without venous reconstruction with prosthesis. Exclusion criteria were surgery before year 2000. Data were descriptive and reverse Kaplan Meier was used for follow-up calculation. The endpoints were the rate of prosthetic reconstruction, the use of anticoagulation and the post-operative outcomes. RESULTS: Fifty - one patients were included with a median duration of follow-up of 54.8 months. The majority of patients were men (56.9%). Median age of the population was 44.1 years. Most of the patients underwent surgery for primary testicular cancer and for sarcoma. Complete IVC resections were performed in 46 (90,2%) patients, 32 having a concomitant prosthetic replacement. Eight patients underwent aortic resection in the same operative time. Postoperative morbidity was 33.3%. Post-operative anticoagulation was done in 24 patients. At 1 month, four patients developed thrombosis in the prosthesis. CONCLUSIONS: IVC resections are feasible and safe. Venous reconstruction and postoperative management were planned according to the preoperative imaging and intraoperative findings. We propose a decision-tree for peri-operative management and anticoagulation.

Analysis of risk factors for post-operative recurrence or progression of intravenous leiomyomatosis.

OBJECTIVE: To analyse the risk factors for post-operative recurrence or progression of intravenous leiomyomatosis and explore the impact of different treatment strategies on patient prognosis. METHODS: Patients with intravenous leiomyomatosis who underwent surgery from January 2011 to December 2020 and who were followed for ≥3 months were included. The primary endpoint was recurrence (for patients with complete resection) or progression (for patients with incomplete resection). Kaplan-Meier survival analysis was used to analyse the factors affecting recurrence. RESULTS: A total of 114 patients were included. The median age was 45 years old (range 24-58). The tumors were confined to the uterus and para-uterine vessels in 48 cases (42.1%), while in 66 cases (57.9%) it involved large vessels (iliac vein or genital vein and/or proximal large veins). The median follow-up time was 24 months (range 3-132). Twenty-nine patients (25.4%) had recurrence or progression. The median recurrence or progression time was 16 months (range 3-60). Incomplete tumor resection (p=0.019), involvement of the iliac vein or genital vein (p=0.042), involvement of the inferior vena cava (p=0.025), and size of the pelvic tumor ≥15 cm (p=0.034) were risk factors for recurrence and progression. For intravenous leiomyomatosis confined to the uterus or para-uterine vessels, no post-operative recurrence after hysterectomy and bilateral oophorectomy occurred in this cohort. Compared with hysterectomy and bilateral oophorectomy, the risk of recurrence after tumorectomy (with the uterus and ovaries retained) was significantly greater (p=0.009), while the risk of recurrence after hysterectomy was not significantly increased (p=0.058). For intravenous leiomyomatosis involving the iliac vein/genital vein and the proximal veins, post-operative aromatase inhibitor treatment (p=0.89) and two-stage surgery (p=0.86) were not related to recurrence in patients with complete tumor resection. CONCLUSION: Incomplete tumor resection, extent of tumor lesions and size of the pelvic tumor were risk factors for post-operative recurrence and progression of intravenous leiomyomatosis.