pseudomyxoma peritonei

腹膜偽粘液腫

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2014/12/24 14:27:01」(JST)

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Pseudomyxoma Peritonei
Classification and external resources
Computed tomographic scan of an abdomen showing pseudomyxoma peritonei with multiple peritoneal masses (arrow) with "scalloping effect" seen.
ICD-10	C48.2
ICD-9	197.6
ICD-O:	M8480
DiseasesDB	31498
eMedicine	med/1795
MeSH	D011553

Pseudomyxoma peritonea is a clinical condition caused by cancerous cells (mucinous adenocarcinoma) that produce abundant mucin or gelatinous ascites. 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. This will result in compression of organs and will destroy the function of colon, small intestine, stomach, or other organs. Prognosis with treatment in many cases optimistic, but the disease is lethal if untreated, with death by cachexia, bowel obstruction, or other types of complications.

This disease is most commonly caused by an appendiceal primary cancer (cancer of the appendix); mucinous tumors of the ovary have also been implicated, although in most cases ovarian involvement is favored to be a metastasis from an appendiceal or other gastrointestinal source. Disease is typically classified as low- or high-grade (with signet ring cells). When disease presents with low-grade histologic features the cancer rarely spreads through the lymphatic system or through the bloodstream.

Pathology and disease classification

There is substantial debate regarding histopathologic classification of pseudomyxoma peritonei. In 1995, Ronnett et al. proposed separating pseudomyxoma peritonei cases into two diagnostic categories: adenoma (disseminated peritoneal adenomucinosis, DPAM) or carcinoma (peritoneal mucinous carcinomatosis, PMCA) with a third category reserved for cases with intermediate features. In this classification system, cases of DPAM were characterized by peritoneal lesions composed of abundant extracellular mucin containing scant simple to focally proliferative mucinous epithelium with little cytologic atypia or mitotic activity (in other words, most cells looked fairly normal and there was no evidence of mitosis which would indicate that cells were rapidly dividing), with or without an associated appendiceal mucinous adenoma. Cases of PMCA were characterized by peritoneal lesions composed of more abundant mucinous epithelium with the architectural and cytologic features of carcinoma (irregular cells, evidence that cells were rapidly dividing, and other criteria), with or without an associated primary mucinous adenocarcinoma. Bradley et al. (2007) argued that continued use of non-malignant terms, i.e., adenoma, for those frequent cases with low-grade features (such as DPAM), is misleading because pseudomyxoma peritonei is a disease state that results from invasion of the abdominal cavity by cells with uncontrolled growth. Bradley states that an adenoma, by definition, is a tumor confined to the appendiceal mucosa with absolutely no evidence of invasion beyond the muscularis mucosae.

The term mucinous adenocarcinoma is used in different contexts depending on the reference material used by the pathologist for disease classification. For example, neoplasms characterized by high-grade features, invasive glands and or signet ring cells, are termed adenocarcinoma in pathology literature. However, some pathologists (e.g., Odze and Goldblum, Surgical Pathology of the GI Tract, Liver, Biliary Tract and Pancreas, 2nd ed.) also use the term mucinous adenocarcinoma when referring to low-grade, well-differentiated tumors lacking high-grade features. Low-grade mucinous adenocarcinoma is used by the American Joint Committee on Cancer and World Health Organization and is nearly or completely synonymous with the DPAM designation. For low-grade mucinous adenocarcinoma, disease may be designated as "benign" because tumors do not invade deeply into tissue and rarely metastasize to parenchyma of organs; this designation may be misleading and confusing to the layperson because pseudomyxoma peritonei is not a harmless condition, fatal if untreated. High-grade or poorly differentiated mucinous adenocarcinoma has a generally poorer prognosis, though surgical treatment with heated intra-peritoneal chemotherapy (HIPEC) is yielding promising outcomes (see surgical treatment).

Etiology

The primary tumour appears to arise from the MUC2 expressing goblet cells and most commonly from these cells in the appendix. The K-Ras (p53) gene may be involved in the oncogenesis. It may be diagnosed with a range of conditions. While the majority of these cases are associated with appendiceal carcinomas, other conditions may also be found, including disseminated peritoneal adenomucinosis (DPAM), peritoneal carcinomas, several mucinous tumors (mucinous adenocarcinoma, mucinous cystadenoma, and mucinous cystadenocarcinoma), as well as other disease states. Other primary sites that have been reported include colon, rectum, stomach, gallbladder, bile ducts, small intestine, urinary bladder, lung, breast, fallopian tubes and pancreas.

Signs and symptoms

Symptoms of pseudomyxoma peritonei may include abdominal or pelvic pain and/or bloating, distension, digestive disorders, weight changes, increased girth and infertility.

Diagnosis

This disease is often discovered during surgery for other conditions, e.g., hernia repair, following which an experienced pathologist can confirm the diagnosis. Advanced stages may present as tumors palpable on the abdomen or distention of the belly ("jelly belly" is sometimes used as a slang term for the condition). Due to the rarity of this disease, it is important to obtain an accurate diagnosis so that appropriate treatment may be obtained from a surgical oncologist who specializes in appendix cancer. Diagnostic tests may include CT scans, examination of tissue samples obtained through laparoscopy, and the evaluation of tumor markers. In most cases a colonsocopy is unsuitable as a diagnostic tool because in most cases appendix cancer invades the abdominal cavity but not the colon (however, spread inside the colon is occasionally reported). PET scans may be used to evaluate high-grade mucinous adenocarcinoma, but this test is not reliable for detecting low-grade tumors because those do not take up the dye which shows up on scans. New MRI procedures are being developed for disease monitoring, but standard MRIs are not typically used as a diagnostic tool. Diagnosis is confirmed through pathology.

Treatment

Treatment is variable, both due to its rarity and to its frequently slow-growing nature. Treatment ranges from watchful waiting to debulking and hyperthermic intraperitoneal chemotherapy (HIPEC, also called intraperitoneal hyperthermic chemotherapy, IPHC) with cytoreductive surgery.

Surgical

The standard of care for mucinous adenocarcinoma with clinical condition PMP involves cytoreductive surgery (CRS) with hyperthermic intraperitoneal chemotherapy (HIPEC, sometimes called intra peritoneal hyperthermic chemotherapy, IPHC) by surgical oncologists who specialize in treating PMP. Some surgeons also apply early post-operative chemotherapy (EPIC) adjunct to surgical cytoreduction and HIPEC. In situations where surgery is not required immediately, patients can be monitored via CT scans, tumor marker laboratory tests, and physical symptoms, to determine when, and if, surgery is warranted. Although some surgical procedures may be rather extensive, patients can and do recover from surgery, and the majority of these patients can and do live productive lives.

In debulking, the surgeon attempts to remove as much tumor as possible. CRS or cytoreductive surgery involves surgical removal of the peritoneum and any adjacent organs which appear to have tumor seeding. Since the mucus tends to pool at the bottom of the abdominal cavity, it is common to remove the ovaries, fallopian tubes, uterus, and parts of the large intestine. Depending upon the spread of the tumor, other organs might be removed, including but not limited to the gallbladder, spleen, and portions of the small intestine and/or stomach. For organs that cannot be removed safely (like the liver), the surgeon strips off the tumor from the surface.

Chemotherapy

Chemotherapy (typically the agent Mitomycin C) may be infused directly into the abdominal cavity after cytoreductive surgery to kill remaining microscopic cancerous tumors and free floating cells. The heated chemotherapy (HIPEC) is perfused throughout the abdominal cavity for an hour or two as the last step in the surgery, or ports are installed to allow circulation and/or drainage of the chemicals for one to five days after surgery (EPIC). EPIC is may be given in multiple cycles for several months after surgery.

Systemic chemotherapy may be administered as additional or adjuvant treatment. Due to the increased availability of new chemotherapies developed for colon and colorectal cancer patients, some patients have experienced stability in tumor growth with systemic chemotherapy. Systemic chemotherapy is reserved for patients with advanced disease, recurrent disease, or disease that has spread to the lymph nodes or distant sites.

This disease may recur following surgery and chemotherapy. Periodic post operative CT scans and tumor marker laboratory tests are used to monitor the disease for any tumor regrowth.

Additionally recent (2003) publications linking the mucin 2 protein overexpression to the cell reproduction has launched research efforts into additional drug treatments.

Epidemiology

The overall incidence is ~1-2 per million per year. It is slightly more common in women than men (male:female ratio = 9:11). The median age at presentation is typically about 50 years with a range of 20–25 years.

History

The first case was described by Carl F. Rokitansky in 1842. Werth in 1884 coined the term pseudomyxoma peritonei, describing it in association with a mucinous ovarian tumour. In 1901 Frankel described the first case associated with a cyst of the appendix.

References

External links

• Pseudomyxoma peritonei at the Open Directory Project

UpToDate Contents

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• 1. 虫垂癌および腹膜偽粘液腫 cancer of the appendix and pseudomyxoma peritonei
• 2. 成人における急性虫垂炎のマネージメント management of acute appendicitis in adults
• 3. 境界悪性卵巣腫瘍 borderline ovarian tumors
• 4. 悪性腹膜中皮腫：疫学、危険因子、臨床所見、診断、および病期分類 malignant peritoneal mesothelioma epidemiology risk factors clinical presentation diagnosis and staging
• 5. 上皮性の卵巣癌、卵管癌、および腹膜癌：病理 epithelial carcinoma of the ovary fallopian tube and peritoneum histopathology

English Journal

• Pressurized Intraperitoneal Aerosol Chemotherapy with Cisplatin and Doxorubicin in Women with Peritoneal Carcinomatosis: A Cohort Study.

• Tempfer CB1, Rezniczek GA2, Ende P2, Solass W3, Reymond MA4.
• Anticancer research.Anticancer Res.2015 Dec;35(12):6723-9.
• AIM: We aimed to assess the objective tumor response (OTR) to laparoscopic pressurized intraperitoneal aerosol chemotherapy (PIPAC) in women with peritoneal carcinomatosis (PC).PATIENTS AND METHODS: We carried-out a retrospective cohort study of women with PC undergoing repeated courses of PIPAC wit
• PMID 26637888

• A Novel Member of Chitinophagaceae Isolated from a Human Peritoneal Tumor.

• Lo AS1, Merrell DS1, Lei H2, Sardi A3, McAvoy T4, Testerman TL5.
• Genome announcements.Genome Announc.2015 Nov 12;3(6). pii: e01297-15. doi: 10.1128/genomeA.01297-15.
• Peritoneal tumors from a rare malignancy, pseudomyxoma peritonei, frequently contain bacteria. Evidence suggests that tumor-associated bacteria contribute to pseudomyxoma peritonei development and/or progression. One unique isolate (PMP191F) was characterized via whole-genome sequencing using the Il
• PMID 26564036

• Glisson capsulectomy for extensive superficial liver involvement in peritoneal carcinomatosis (with video).

• Dagbert F1, Passot G2, Glehen O2, Bakrin N3.
• Journal of visceral surgery.J Visc Surg.2015 Nov;152(5):332-3. doi: 10.1016/j.jviscsurg.2015.08.002. Epub 2015 Oct 21.
• PMID 26476675

Japanese Journal

• 右胸腔内浸潤を合併した腹膜偽粘液腫に対し,完全減量手術を2期的に行った1症例

• 伊藤 大真,安間 記世,豊永 庸佑 [他]
• 麻酔 64(6), 655-659, 2015-06
• NAID 40020498057

• Reduced Port Surgeryで切除した高度炎症を伴う虫垂中に発見された潜在性虫垂粘液嚢胞腺腫の１例

• 鈴木 宏光,木村 紘爾,木下 茂喜,岡野 和雄
• 岡山医学会雑誌 126(3), 223-226, 2014-12-01
• … At one year and a half after surgery, there was no evidence of the development of pseudomyxoma peritonei. …
• NAID 120005525325

• 腹膜偽粘液腫の診断と治療 (特集 保存版 消化器外科領域の希少悪性腫瘍) -- (その他の希少悪性腫瘍)

• 清崎 浩一,斉藤 正昭,力山 敏樹
• 外科 = Surgey : 臨床雑誌 76(10), 1137-1139, 2014-10
• NAID 40020215054

Related Pictures

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★リンクテーブル★

リンク元	「腹膜偽粘液腫」「gelatinous ascites」

「腹膜偽粘液腫」

　　[★]

英

peritoneal pseudomyxoma

ラ

pseudomyxoma peritonei

関

• 粘液性ないしゼラチン様物質を産生する腫瘍が腹膜播種した結果、腹腔内にブドウ様の嚢胞や腫瘤、粘液が充満した状態。(SSUR.472)
• 中年の女性に多い
• 病因：

• (1)卵巣の粘液嚢胞、虫垂の粘液瘤などの破裂、(2)粘液産生癌の腹膜播種、(3)良性粘液の右方の腹膜面での異所性発育。(SSUR.472)
• 大腸・虫垂の粘液癌、卵巣の粘液嚢胞腺癌・粘液嚢胞腺腫の穿孔により生じうる(YN.A-115)

• 予後：不良(ゼリー状の腹水は腹腔穿刺によって吸引できないため、開腹手術となるが、繰り返し貯留して次第に腸管の通過障害をきたし衰弱していく)

国試

• 102D029、099G030

「gelatinous ascites」

　　[★] ゼリー状腹水

関

pseudomyxoma peritonei