Chaoji Zhang

Intravenous Leiomyomatosis With Intracardiac Extension

Intravenous leiomyomatosis with right intracardiac extension is rare. Surgical treatment of the tumor is still controversial because of the high postoperative risk of morbidity and mortality. We describe a series of 5 patients with these lesions who underwent elective operations with different strategies, including one-staged or two-staged resections and cardiopulmonary bypass with beating heart, cardioplegic arrest, or deep hypothermic circulatory arrest. We believe that this report represents one of the largest series of patients encountered in a single institution. In conclusion, radical resection is always possible and the outcomes are satisfactory with planned surgery.

Pulmonary Embolization as the Primary Clinical Manifestation of Intravenous Leiomyomatosis With Intracardiac Extension

A 47-year-old woman was admitted to our department with a 6-month history of worsening dyspnea. She had undergone a hysterectomy with right salpingooophorectomy for benign leiomyoma 7 years ago. An echocardiogram revealed a mobile mass extending from the inferior vena cava into the right heart (Fig 1). Computed tomography pulmonary angiography revealed filling defects consistent with pulmonary embolus in multiple lobe segmental branches of the right pulmonary artery (Fig 2A). Computed tomography venography showed a hypodense

Primary cardiac pheochromocytoma with multiple endocrine neoplasia.

PurposeCardiac pheochromocytoma with multiple endocrine neoplasia syndrome (MENS) is rare. We present a rare case of concurrent cardiac pheochromocytoma and pituitary adenoma, a rare variant of the MEN syndromes and a review of the literature with special emphasis on diagnosis and treatment.MethodsDifferent from the single MENS type I or type II, Variant MENS’ symptoms and signs are so nontypical that it is easy to make a misdiagnosis or missed diagnosis. One patient with variant MENS was treated surgically and relevant case data were collected.ResultsThe perioperative course was uneventful. At 24-month follow-up, catecholamine levels returned to normal and no symptoms of pheochromocytoma was found.ConclusionsCardiac pheochromocytoma with Multiple Endocrine Neoplasia can be treated by operation with good prognosis. Surgical removal to provide relief or effective control of symptoms is the treatment of choice.

Surgical treatment of pulmonary artery aneurysm: an institutional experience and literature review

OBJECTIVES The occurrence of pulmonary artery aneurysm (PAA) is extremely rare in the clinical setting. Careful treatment should be considered because of the possibility of fatal complications including rupture, dissection, pulmonary embolism and heart failure. Our goal is to contribute a better understanding of this disease and its treatment. METHODS Information on patients diagnosed with PAA was retrieved from the institutes database. The detailed data including aetiology, clinical presentation, diagnostic methods, operation methods and long-term outcomes of the surgical cases were outlined and analysed. RESULTS In total, 21 patients were diagnosed with PAA in Peking Union Medical College Hospital from 1980 to 2015, among whom 5 patients received surgical treatment, including 2 patients with giant PAAs. The complete remission rate of surgical cases was 80%, and the average postoperative hospital stay was 8.5 ± 1.29 days. One postoperative death occurred due to distributive shock. CONCLUSIONS PAAs must be seriously classified by aetiology to be treated appropriately. Patients with giant-size PAAs, and those with pulmonary hypertension, anatomical anomalies, and rapid growth and compression of neighbouring critical structures, are proper candidates for surgery. Surgical options include aneurysm repair and replacement with allogeneic/synthetic grafts, depending on the situation. Additionally, the correction of associated abnormalities should be performed simultaneously during surgery. Surgical outcomes are effective, and long-term prognoses are satisfactory.

Different surgical strategies of patients with intravenous leiomyomatosis.

Abstract Intravenous leiomyomatosis (IVL) is a rare benign tumor. The study aimed to assess outcomes of patients treated surgically for IVL. Between November 2002 and January 2015, 76 patients were treated for IVL. The stage of IVL was evaluated preoperatively by echocardiography and enhanced computerized tomography (CT) scan, and graded into 4 stages according to intravascular tumor progression. We recorded age, lower limb edema before surgery, surgical parameters, and hospitalization expenses. Patients were followed up every 6 months and tumor recurrence was assessed by CT and ultrasound. Patients were followed up for a mean of 4.5 ± 2.5 years (range 1–13 years) and there was no operative, hospital, or long-term mortality or were lost to follow-up. The rate of lower extremity edema, amount of blood loss, postoperative transfusion, length of intensive care unit (ICU) stay, postoperative hospitalization, and hospitalization expenses differed significantly between patients at different presurgery stages. Tumors recurred in 4 of 7 patients with stage I IVL that opted for surgery that preserved the ovaries and uterus. No recurrence was observed in patients graded stage II or more, in all of which the uterus and ovaries were removed. Recurrence was observed in only 4 of 76 cases of IVL, all of whom opted for surgery that spared the ovaries and uterus. Different surgical strategies should be decided based on the staging to completely remove the tumor and ensure the safety of patients. Removal of both ovaries is necessary for inhibiting tumor growth and avoiding recurrence.

Innominate Pseudoaneurysm Subtotally Compressing the Trachea as a Result of Blunt Trauma

Blunt traumatic innominate pseudoaneurysm is rare, and coexisting airway distress is even rarer. We describe a case of innominate pseudoaneurysm that subtotally compressed the trachea in a 45-year-old man. The patient also had bovine-type arch anatomy. He experienced exacerbated respiratory distress on anesthesia induction. A cardiopulmonary bypass (CPB) circuit was immediately established through the femoral vessels. The aortic arch was replaced with a branched graft under circulatory arrest and antegrade cerebral perfusion. The pseudoaneurysm was eliminated and airway compression was completely relieved. The patient fully recovered without major complications. The unique feature of this case is its association with airway compression, which is uncommon but potentially lethal.

Pericardial Tamponade Secondary to Castleman's Disease

24-year-old man was admitted to a peripheral local hospital, providing low-level care with a 6-day istory of progressive cough, fatigue, and dyspnea, which ad worsened over the preceeding 6 hours with addiional symptoms of orthopnea and hypotension. A comuted tomographic scan revealed large pericardial and ilateral pleural effusions. The right pleura were aspiated of 1,000 mL of a yellow transudate with improveent in symptoms. A week later the pericardium was rained by a more experienced visiting physician with urther relief of symptoms. The cytology of fluid was egative for malignancy. The patient was then transerred to our institution, which provides a higher level of are. Laboratory findings demonstrated a mild anemia, levated sedimentation rate, hypergammaglobulinemia, nd high C-reactive protein. Exudative drainage of aproximately 150 mL of exudative fluid continued from the ericardium. Echocardiography revealed excellent left entricular function, a whole-body positron emission omography/computed tomography scan (Fig 1) demon-

Primary Cardiac Pheochromocytoma Involving Both Right and Left Atria

Pheochromocytomas are catecholamine-producing paragangliomas derived from the neural crest. Primary cardiac pheochromocytoma is extremely rare. We describe a case of cardiac pheochromocytoma involving both right and left atria in a middle-aged woman. Metaiodobenzylguanidine (MIBG) scintiscan results were normal, but results from an octreotide scan were abnormal. The tumor was successfully resected using cardiopulmonary bypass (CPB).

Outcomes of Surgery for Functional Cardiac Paragangliomas: A Single-center Experience of 17 patients

Objective: Cardiac paragangliomas are rare neuroendocrine tumors. Early surgical treatment improves clinical symptoms and prolongs survival. We review our experience in 17 patients who underwent surgical resection for functional cardiac paraganglioma. Methods: Seventeen patients underwent surgery for functional cardiac paraganglioma from 2004 to 2017 were identified. Clinical data and long‐term outcomes were extracted and analyzed. Results: All 17 patients with cardiac paraganglioma (11 males) with a median age of 35 years (range, 11 to 51 years) were hormonally functional and underwent operations. A 24‐hour urine catecholamine assay documented elevation of norepinephrine, epinephrine, and dopamine. Tumors were determined with octreotide scintiscan in all 17 patients and metaiodoben‐zylguanidine scintigraphy in 10 of 15 patients. Tumor sites were right atrioventricular groove in 4 patients, between the main arteries in 10 patients, and interatrial groove in 4 patients. All patients underwent complete resection. Concomitant surgeries were: structural reconstruction in 16 patients (94%) and coronary artery bypass graft in 8 patients (47%). One patient had a history of incomplete resection of an intra‐atrial tumor. One patient died postoperatively. Operative mortality is 5.8%. During follow‐up (mean, 6.5 years; range, 1.5 to 14.2 years), 16 patients are alive in functional class I or II. Two patients developed tumor recurrence and 14 remained symptom‐free with normal urinary catecholamines. Conclusions: With a multidisciplinary treatment, early diagnosis, complete resection for the tumor along with complex reconstruction is achievable for most patients, and it offers promising long‐term survival.

Surgical treatment for intravenous-cardiac leiomyomatosis

OBJECTIVES There are few published studies on the rare disorder of intravenous-cardiac leiomyomatosis (IVCL). This study aimed to propose an individualized strategy for surgical treatment of IVCL. METHODS In this retrospective study, we reviewed 50 patients who had undergone IVCL removal from November 2002 to October 2017 in our hospital. IVCL was classified as Type A-E according to the extent and size, with Type E being the most severe. Clinical manifestations, surgical features and follow-up data were analysed. RESULTS Of the 50 patients in this series, 8 had Type A IVCL, 8 Type B, 29 Type C, 2 Type D and 3 Type E IVCL. One-stage removal of IVCL was performed via laparotomy without cardiopulmonary bypass (CPB) in the 8 patients with Type A, 1-stage tumour resection via sternolaparotomy under deep hypothermic arrest in 7 of the 8 patients with Type B and IVCL removal via sternolaparotomy under CPB, with 27 also under deep hypothermic arrest, in all 29 patients with Type C. Sixteen of the patients with Type C IVCL underwent staged procedures, 13 a 1-stage procedure and 21 required hepatic mobilization. All patients with Type C or E cases underwent 1-stage tumour removal via sternolaparotomy under deep hypothermic arrest. All 50 patients survived surgery. IVCL was confirmed postoperatively by histology. Ten patients had residual tumours; 9 of which did not progress. No deaths occurred during 47.8 ± 38.4 (range 1-177) months of follow-up. CONCLUSIONS The only known curative treatment for IVCL is surgery. Herein, we present an individualized strategy for selecting surgical treatment.