Health, general cat scans usage ct imaging myxoma care and treatment case studies diagnosis. Cancers of the appendix are an extremely rare and deadly disease found in the abdominal region. Pseudomyxoma peritonei pmp also known as jelly belly or gelatinous ascites or false mucinous tumor of the peritoneum is a rare complication with an. Radiological case, case study by applied radiology. The patient underwent omental biopsy and the final pathology diagnosis was lowgrade. Pseudomyxoma peritonei radiology reference article. Ovarian carcinoma must be included in the differential diagnosis to explain the pseudomyxoma peritonei at least it used to be included.
As the mucin producing cells in pseudomyxoma peritonei are poorly adherent, they are easily dislodged by peristaltic movement and adhere at sites of relative stasis. Pseudomyxoma peritonei pmp is a rare clinical condition, where copious mucinous ascites accumulate in the peritoneal cavity due to dissemination of mucinproducing tumor. Although there is increasing knowledge of pmp, it remains a diagnostic challenge because of its rarity and lack of differential diagnostic symptoms. Extensive lobular formations of low attenuation masses hu 25 surround and encase the liver and spleen scalloping the surfaces. Ultrasonography and computed tomography in pseudomyxoma. The presence of cells in the mucin, either inflammatory or neoplastic, distinguishes it from simple acellular mucus ascites caused by mucinous spillage. Pdf we report a rare case of pseudomyxoma retroperitonei in a. Extraabdominal spread of pseudomyxoma peritonei is a rare occurrence, with few reports in the medical literature. Mucocele of the appendix and pseudomyxoma peritonei ah dachman, je lichtenstein, ac friedman american journal of roentgenology. Pseudomyxoma peritonei pmp is a rare condition resulting from mucinproducing tumors that have disseminated into intraperitoneal implants and mucinous ascites. The cause was a mucinproducing tumor of the appendix. Intraoperative diagnosis is also tricky because the inflammation of the appendix often hides the tumor.
Theres two hospitals that specialise in treatment for pseudomyxoma peritonei in the uk. Thus, pseudomyxoma peritonei is a mucoid tumour of the peritoneum that resembles but is not, myxoma. Three patients with pseudomyxoma peritonei were found to have quite different sonographic and ct features from those described previously. Pdf on mar 1, 1989, p s lawate and others published ct and sonographic. Before the advent ofcomputed tomography ct ofthe body, the radiographic findings ofpseudomyxoma were. Myxoma is instead a rare tumour of the primitive connective tissue. Pseudomyxoma peritonei refers to syndrome of progressive intraperitoneal accumulation of mucinous ascites related to a mucinproducing neoplasm. Pseudomyxoma peritonei develops at a variable rate, but may grow at a slower rate indolent than other malignancies within the abdomen. Pseudomyxoma peritonei is a common manifestation of mucinous adenocarcinoma. Pdf mucocele of the appendix and pseudomyxoma peritonei. Nevertheless, pseudomyxoma peritonei is a useful term for describing a distinctive clinical picture produced by welldifferentiated mucinous neoplasms in. Because of this disseminating, yet nonmetastasizing, behavior, pmp attracts much interest from surgical oncologists in that aggressive locoregional therapy can give the opportunity of long survival. Pseudomyxoma peritonei pmp is an unusual clinical entity characterised by mucinous ascites eventually leading to gross abdominal distension. Axial contrastenhanced ct images of the abdomen and pelvis demonstrate lobulated, lowattenuation soft tissue masses scalloping the borders of the liver, spleen and along mesentery.
These are recommended by the national institute for health and care excellence nice. Pdf pseudomyxoma peritonei is due to diffuse involvement of the peritoneal cavity with mucinous material. Pseudomyxoma peritonei nord national organization for. The cytology of five cases of pseudomyxoma peritonei is described. Features on ct scan and the extensive scalloping of the liver suggest rupture of a mucinous neoplasm resulting is pseudomyxoma peritonei. Pseudomyxoma peritonei is defined as the presence of abundant mucinous material on peritoneal surfaces. Journal of clinical imaging science dilated appendix.
Pleural extension of mucinous tumor in patients with. The characteristic mucinous ascites is composed of acellular mucin and a variable amount of neoplastic epithelial cells. Pseudomyxoma peritonei secondary to mucocele of the. Pseudomyxoma peritonei pmp is a rare condition that usually starts with a tumor in your appendix though the tumor also can be in your bowel, bladder, or ovaries. Miller dl, udelsman r, sugarbaker ph 1985 calcification of pseudomyxoma peritonei following intraperitoneal chemotherapy. There is widespread seeding of the peritoneal and omental surfaces with a heavy cancerous glaze. For more information, click on the link if you see this icon. We report a rare case of pseudomyxoma retroperitonei in a 58yearold woman with a past history of severe appendicitis. Pseudomyxoma peritonei pmp is a mucinous tumour of the appendix. The patient underwent omental biopsy and the final pathology diagnosis was lowgrade mucinous neoplastic gastrointestinal type epithelium. Soyearold man with pseudomyxoma peritonei of appendiceal origin who. Matsuoka y, ohtomo k, itai y, nishikawa j, yoshikawa k, sasaki y 1992 pseudomyxoma peritonei with. Pseudomyxoma peritonei pmp is an uncommon borderline malignancy generally arising from a perforated appendiceal epithelial tumour. Pseudomyxoma peritonei pmp is a clinically distinctive form of malignancy that typically arises from the appendix and is characterized by bulky accumulation of mucinous tumor deposits in the peritoneal cavity.
Asymptomatic appendiceal neoplasms may be discovered incidentally. Pdf ct and sonographic features of pseudomyxoma peritonei. Traditionally pmp has been treated with serial debulking procedures. Pseudomyxoma survivor is a global organisation headquartered in the uk, dedicated to supporting those affected by pseudomyxoma peritonei pmp, appendix cancer and other peritoneal surface malignancies and raising awareness. A 71yearold male patient presented with intermittent diarrhea and loss of appetite during the last two months, without any other. Pseudomyxoma pleurii and peritonei secondary to sigmoid. Pseudomyxoma peritonei pmp is a rare intraperitoneal tumor, characterized by disseminated intraperitoneal tumor implants on peritoneal surfaces.
Pseudomyxoma peritonei is a relatively rare and poorly understood condition in which mucus accumulates within the peritoneal cavity. Pseudomyxoma peritonei is one of the complications of mucin secreting tumours arising from appendix or ovary. Pseudomyxoma peritonei what every radiologist should know. The biological characteristics of classical pmp are controversial in that demonstrable invasion at the primary site may be. Imaging findings of urachal mucinous cystadenocarcinoma. Pseudomyxoma extraperitonei is rare lesion resulting from the rupture of an appendiceal mucocele into the extraperitoneal tissues. Pseudomyxoma peritonei an overview sciencedirect topics. Your treatment depends on the size of the cancer and your general health. Pseudomyxoma peritonei pmp is a clinical condition caused by cancerous cells mucinous adenocarcinoma that produce abundant mucin or gelatinous ascites. Pseudomyxoma peritonei pmp is a mucinous tumour of the appendix that spreads into the peritoneal cavity in the form of gelatinous deposits. Interventional radiology strategies in the treatment of. Although this tumor is only superficially invasive and does not metastasize, it is a fatal disease.
We report a case of aggressive disease with an unusual clinical presentation and we analyze current data on diagnosis and management of pmp. Fisher2 case reports pseudomyxoma penitonei isararecomplication ofmucocele onadenocancinoma oftheappendix andcystadenomaorcystadenocarcinoma oftheovary 14. Appendix cancer pseudomyxoma peritonei pmp download pdf copy. The disseminated neoplastic cells produce mucin, which leads to the characteristic mucinous ascites 1.
It is not a complete diagnosis in itself, because the prognosis depends on the nature of the causative lesion. The main treatments for pseudomyxoma peritonei pmp are surgery and chemotherapy. Mucocele of the appendix is rare and represents only the tip of the iceberg of underlying benign and malignant pathological processes. A nasogastric tube is in situ with the tip projected within the anterior gastric body. The preoperative diagnosis is essential to differentiate appendiceal mucocele from acute appendicitis as the treatment. The doctor closely monitors your cancer in case you need treatment in the future.
For language access assistance, contact the ncats public information officer. Any information contained in this pdf file is automatically generated from. To describe percutaneous maneuvers to treat the unusual entity symptomatic pseudomyxoma peritonei pmp. If you have problems viewing pdf files, download the latest version of adobe reader. Pseudomyxoma peritonei is a rare disease characterized by a large amount of mucinous ascites with peritoneal and omental implants. The tumors cause fibrosis of tissues and impede digestion or organ function, and if left untreated, the tumors and mucin they produce will fill the abdominal cavity. Ascites is a nonspecific ct scan finding in pseudomyxoma peritonei. Modalities like mdct radiological scores have been shown to have. The extraabdominal spread of pmp is exceptionally rare, with few. Ultrasound us revealed a a large, highly echogenic mantle consisting of numerous tiny cysts, b echogenic ascites which was actually gelatinous in nature, or c multiple indentations of the bowel by semisolid masses. Optimal treatment involves a combination of cytoreductive. Some authors oppose use of adenomucinosis and borderline as misleading, because lesions may have aggressive biologic behavior.
The patients predisposing pathology for pseudomyxoma peritonei is unknown. Case report acta radiologica imaging findings of urachal mucinous cystadenocarcinoma associated with pseudomyxoma peritonei m. It is important to understand that pseudomyxoma peritonei represents a spectrum of disease. A case report volume 2 issue 4 2015 abdulhamid g1, tamimi ah2, laswar kn2, shukry s 1, alahdel f and yassin s 1national oncology center, yemen 2department of surgery, university of aden, yemen corresponding author. There is widespread seeding of the peritoneal and omental surfaces with a heavy cancerous. Pseudomyxoma peritonei of appendiceal origin is due to invasion or rupture of the appendix from a mucinsecreting appendiceal tumor. The incidence of pmp is believed to be approximately out of a million per year. It has defied stable pathologic categorization ever since first being described in the late 19th century. Mucocele of the appendix and pseudomyxoma peritonei.
Clinical and radiological features of pseudomyxoma peritonei. Nonetheless, due to its indolent nature, it is usually discovered at an advanced stage and severely impacts quality of life. Mucoceles from either benign or malignant mucinous neoplasms represent the majority of appendiceal tumors detected at imaging but are the least likely to manifest as appendicitis. Clinically pseudomyxoma peritonei presents no inconvenience to the patient until enlargement of the abdomen occurs, as simple rupture of t,he ovarian tumor does not, usually produce any noticeable symptoms. Pseudomyxoma peritonei is a relatively rare and poorly understood. Case of the week 389 on awardwinning, radiologic teaching site for medical students and those starting out in radiology focusing on chest, gi, cardiac and musculoskeletal diseases containing over 200 powerpoint lectures, quizzes, handout notes, interactive material, most commons lists and pictorial differential diagnoses. The imaging showed a multicystic mass similar to pseudomyxoma peritonei. Used for complete redistribution pattern as seen in lowgrade mucinous adenocarcinoma of the appendix or pseudomyoxma peritonei. Pseudomyxoma peritonei postgraduate medical journal. Four patients with pmp were treated by interventional radiology techniques that included large catheters 2030 fr alone n 3, multiple catheters n 4, and dextran sulfate as a catalytic agent through smaller catheters n 1. Pseudomyxoma peritonei pmp is an uncommon surgical entity.
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