Retroperitoneal neoplasms are rare and easily misdiagnosed. Learn vocabulary, terms, and more with flashcards, games, and other study tools. Large retroperitoneal mass in a young female patient. They are mostly benign in nature, more commonly occurs in adult females between 2050 years age with a male to female ratio of 2. This makes the grade of the cancer a more important factor when finding out the stage of your cancer. Retroperitoneal liposarcoma frequently recurs asymptomatically within 2 years, usually within 6 months2 years, of the initial surgical resection. Special problems encountering surgical management of large. This tumor can affect people of any age but most often shows up between the ages of 30 and 50.
Our pdf merger allows you to quickly combine multiple pdf files into one single pdf document, in just a few clicks. The ct features of recurrent liposarcoma are similar to those of the initial manifestation. These tumors are often discovered incidentally during imaging studies performed for other reasons. Therefore, local recurrence after surgical resection is. They synthesize, store and secrete catecholamines because of which they may present. In 60% of cases, they secrete hormones and are classified as pheochromocytomas. To determine whether mri feature analysis can differentiate benign retroperitoneal extraadrenal paragangliomas and schwannomas. Jul 16, 20 the paraganglioma is a rare tumor associated with high morbidity and mortality when the diagnosis is made intraoperatively. Case note retroperitoneal nonfunctioning paraganglioma p aragangliomas are extraadrenal chromaffin tumours1 that develop at the expense of neuroectodermal cells of the autonomous nervous system. Surgery was 8 or 9 hours during which i also had a bowel resection, a. Retroperitoneal tumor, mucinous cystadenocarcinoma, mural nodule introduction primary retroperitoneal mucinous cystadenocarcinoma prmc is an extremely rare tumor.
The first case was presented in 1965 1, with only 66 cases, including our case, having been described in the english literature to date 28. Ctabdomenreveals retroperitoneal mass arise below the body and tail of pancreas and anterior to the left kidney. Primary neuroendocrine tumor of the retroperitoneum. Joana santos2, rosa cardoso3, sofia carvalho3, sonia vilaca 4, manuela vasconcelos.
Paragangliomas are rare with multicentricity being more common in patients with familial history. The lesion has heterogenous consistency and enhancing heterogeneously after administration of iv contrast. Retroperitoneal sarcomas are commonly seen in the 5th and 6th decades of life. To our knowledge this is the first report of this kind of disease in the literature.
They arise from embryonic neural crest cells and are composed mainly of chromaffin cells located in the paraaortic sympathetic chain. Among these imaging methods, the primary methods of diagnosis are anatomic imaging techniques, including ct and mri, and functional imaging techniques with metaiodobenzylguanidine mibg scintigraphy. Liposarcomas rarely arise in the mesentery or peritoneum 1, 2. Of 253 resections, 22 9% were retroperitoneal and were selected for further study. In addition, most patients are diagnosed between 30 and 45 years of age andersen et al. A scanning microphone is moved over the part of the body that is being scanned, gel is placed on the skin. Clinical presentation and treatment outcomes in two patients. Early recognition of the characteristic symptoms of headache, palpitations, and diaphoresis in a patient with hypertension and prompt appropriate intervention can minimize the morbidity associated with such tumors and prevent a potentially fatal outcome. Retroperitoneal extraadrenal paraganglioma eap are relatively rare neuroendocrine tumors. Last april i was diognosed with retroperitoneal liposarcoma. Cystic retroperitoneal lesions radiology reference article. We report a case of a 42 year old female presenting with abdominal pain who had a retroperitoneal tumour situated at the aortic bifurcation. Oct 23, 2009 i had a retroperitoneal liposarcoma, attached to my right lower quadrant rectus muscle, removed on 11411. Retroperitoneal sarcomas ppt suny downstate medical center.
Solid neoplastic retroperitoneal mass fetus in fetus fetal skull with hairs teratoma 8. Feb 15, 2015 retroperitoneal fibrosis retroperitoneal fibrosis is an uncommon collagen vascular disease of unknown cause that can mimic a retroperitoneal tumor. Retroperitoneal paraganglioma with metastasis to the. Clinically and histologically, these tumors are similar pancreatic and ovarian mucinous neoplasms. Retroperitoneal fibrosis is typically idiopathic 70% of cases and is likely autoimmune in origin. Extraadrenal paraganglioma of the retroperitoneum with metastasis to the vertebra is very rare. A twentyfiveyearold female presented with a large retroperitoneal mass. Diagnosis and management of retroperitoneal softtissue sarcoma. With pdf merger you can merge your multiple pdf files to a single pdf file in matter of seconds. Multiple retroperitoneal paragangliomas springerlink.
Cystic retroperitoneal lesions radiology reference. A rare case of retroperitoneal ganglioneuroma incidentally found during an annual health examination is presented. Unfortunately, surgery is curative in only a minority of patients, and liposarcoma shows a high rate of local. Schwannomas have true capsules composed of epineurium. Retroperitoneal laparoscopic management of paraganglioma. Physical examination and routine laboratory studies gave normal results. Surgical management of primary retroperitoneal tumors. The only effective treatment for a retroperitoneal liposarcoma is surgical resection 3, 4. I had a retroperitoneal liposarcoma, attached to my right lower quadrant rectus muscle, removed on 11411. A complete physical examination and thorough evaluation of the individuals medical history. Preoperative diagnosis helps to plan treatment and prevent complications.
Clinically, patients with a retroperitoneal paraganglioma often present with back pain or a palpable mass. There is well defined heterogenous soft tissue lesion at the right side of the retroperitoneal cavity located at the posterolateral aspect of the right kidney measuring 78mm length x 54mm width x 55mm ap diameter. Paragangliomas are tumors that arise from extraadrenal medullary neural crest derivatives. Is essential to rule out the existence of paraganglioma in all retroperitoneal tumors, even in asymptomatic and hemodynamically stable patients. Composite paragangliomaganglioneuroma in the retroperitoneum article pdf available in world journal of surgical oncology 71. Primary retroperitoneal paraganglioma simulating a pancreatic. Visible mass occupying the epigastric and left lumbar region. Learn indepth information on welldifferentiated liposarcoma, its causes, symptoms, diagnosis, complications, treatment, prevention, and prognosis. Extraadrenal paragangliomas have nearly identical imaging features, including a homogeneous or heterogeneous hyperenhancing softtissue mass at ct, multiple areas of signal void interspersed with hyperintense foci saltandpepper appearance within tumor mass at mri, and an intense tumor blush with enlarged feeding arteries at angiography. Retroperitoneal fibrosis retroperitoneal fibrosis is an uncommon collagen vascular disease of unknown cause that can mimic a retroperitoneal tumor. Your story matters citation xu, weifeng, hanzhong li, zhigang ji, weigang yan, yushi zhang, xuebin zhang, and qian li. This was not attached to any vital organs except for a small attachment to my small intestine and they removed. If it forms in the retroperitoneal space, the tumor may not cause any symptoms for a long period of time, until it grows to a large size and compresses the adjacent organs in the abdomen.
Nakakura division of surgical oncology ucsf department of surgery ucsf hellen diller family comprehensive cancer center san francisco, ca may 18, 20 52yearold man with testicular ca 30 yrs ago, sp orchiectomy, ebrt retroperitoneal sarcoma case presentation. Since retroperitoneal paragangliomas are rare, the behavior and treatment outcomes of this type of tumor remain unclear. Paragangliomas are extraadrenal tumors of the autonomic nervous system and may be found within the skull base, neck, chest, and abdomen. In essence, masses in the retroperitoneum can be categorized as only one of three candidate entities. Tumor size was 10 x 10 x 9 cm, weight of tumor was just under 2 lbs. An 18yearold, unmarried woman presented with pain and progressively increasing swelling in right side of the abdomen of 4 months duration.
We describe a case of primary retroperitoneal granulosa cell tumor, which, to our knowledge, has been previously reported in few cases. It is fascinating and challenging to medical fields because it carries a high risk of morbidity and mortality if it is undiagnosed or untreated. Retroperitoneal schwannomas are rare, usually benign tumors that originate in the neural sheath and account for only a small percentage of retroperitoneal tumors. Most of the retroperitoneal neoplasms are of mesodermal origin, with liposarcomas, leiomyosarcomas, and malignant fibrous histiocytomas making up more than 80% of these tumors. Cystic retroperitoneal lesions can carry a relatively broad differential, which includes. They usually progress to a very large size before prompting. Nov 12, 2015 paragangliomas are rare neoplasms arising from cells of the primitive neural crest. Rpss lack specific symptoms or laboratory findings.
The tumor is often slow growing and noncancerous benign. Understanding retroperitoneal sarcomas sarcomaorgu understanding retroperitoneal sarcomas 7 most retroperitoneal sarcomas are more than 5cm in size and deep in the body. Here, we present an oroginal case of paraganglioma of the retroperitoneum with metastasis to the abdominal vertebra in a 42yearold female patient who was successfully treated by complete removal of the tumor and. Composite paraganglioma ganglioneuroma in the retroperitoneum article pdf available in world journal of surgical oncology 71. Local recurrence of retroperitoneal paraganglioma treated laparoscopically ryuta watanabe, md, akira ozawa, md, tokuhiro iseda, md department of urology, matsuyama shimin hospital, ehime, japan all authors. It is a dedifferentiated cancer, grade 3 high grade. Aliposarcoma is a malignant tumor of mesenchymal origin that is one of the most common primary neoplasms in the retroperitoneum. They are usually located in the head and neck but can be found in various body sites, including the chest cavity, abdomen, pelvis and bladder. Cancerous forms represent 20%50% of cases and are characterized by the occurrence of ganglionic or distant metastases in 30% of cases. Spectrum of retroperitoneal and genitourinary paraganglioma. These tumours are often difficult to diagnose and treat.
Ines a, haifa b r, fedi s, welid z, ali majdoub, retroperitoneal secretant paraganglioma. A 64yearold female presented with large intraabdominal mass and vague abdominal pain. Retroperitoneal tumor is a rare but diverse group of neoplasms that arise from the retroperitoneum, comprising about 10% of all soft tissue tumors. Mar 28, 20 extraadrenal paraganglioma of the retroperitoneum with metastasis to the vertebra is very rare. Welldifferentiated liposarcoma may be diagnosed by using the following tools and procedures. It is rare to find a schwannoma in the retroperitoneal cavity and accounts for 0. Extra adrenal retroperitoneal paragangliomas are extremely rare neuroendocrine neoplasms with an incidence of 28per million. Paraganglioma of the kidney seldom occurs and is rarely malignant. A single institute experience the harvard community has made this article openly available. The exact incidence of retroperitoneal paragangliomas is unknown, although males are typically affected more frequently than females.
Stage 1 means the cancer is low grade, small less than. Jan 09, 2019 how is welldifferentiated liposarcoma diagnosed. Apr 28, 2016 solid neoplastic retroperitoneal mass fetus in fetus fetal skull with hairs teratoma 8. Workup included history and physical exam, imaging, biopsy, colonoscopy, and gynecologic exam. Retroperitoneal sarcoma surgical management of soft tissue. Thus preoperative assessment, intraoperative monitoring, surgical skill. Gross pathology the mass already bisected in two different containers measuring 9x6. Experience at a north indian tertiary care center santosh kumar1, gautam ram choudhary1, shivanshu singh1, seema prasad2, shrawan kumar singh1, anil bhansali3, sanjay bhadada3, pinaki dutta3. A read is counted each time someone views a publication summary such as the title, abstract, and list of authors, clicks on a figure, or views or downloads the fulltext. Primary retroperitoneal paraganglioma simulating a. Their proximity to vital structures especially vascular makes resection difficult.
These features were compared between two types of tumours by. Several radiologic imaging and nuclear imaging techniques are currently available to evaluate extraadrenal paragangliomas of the body. Mr imaging features of benign retroperitoneal paragangliomas. After surgical resection, the mass was determined to be a primary retroperitoneal mucinous tumor prmt. When presenting within the abdominal cavity, they may arise as a primary retroperitoneal neoplasm and can mimic vascular malformations or other conditions related to specific retroperitoneal organs such as the pancreas, kidneys, or adrenals. Retroperitoneal paraganglioma a rare cause of arterial. Radiological studies, such as xray, ct, mri, or ultrasound scan of the affected region. This free online tool allows to combine multiple pdf or image files into a single pdf document. Non neoplastic retroperitoneal mass solid cystic retroperitoneal fibrosis ormonds disease hematoma urinoma pseudocyst 10. She had a history of hysterectomy and bilateral salpingooopherectomy 22 years ago for large uterine leiomyoma.
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