Pseudomyxoma peritonei differential diagnosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Nima Nasiri, M.D.[2]Parminder Dhingra, M.D. [3]

Overview

Pseudomyxoma peritonei must be differentiated from peritoneal carcinomatosis without mucinous ascites, peritoneal sarcomatosis, and peritonitis.

Differential Diagnosis

Pseudomyxoma peritonei must be differentiated from:

• Peritoneal carcinomatosis without mucinous ascites
• Peritoneal sarcomatosis
• Peritonitis

Differentiating pseudomyxoma peritonei from other diseases on the basis of [symptom 1], [symptom 2], and [symptom 3]

• On the basis bloating, abdominal distention, infertility and hernia , must be differentiated from [disease 1], [disease 2], [disease 3], [disease 4], [disease 5], and [disease 6].

Diseases	Clinical manifestations			Para-clinical findings						Gold standard	Additional findings
	Symptoms		Signs
				Lab Findings		Imaging			Histopathology
	Symptom	Constitutional symptoms	Physical exam	CBC	Immunohistochemistry	CT Scan	MRI	Ultrasound
Pseudomyxoma peritonei	Bloating Abdominal distention	Negative	Abdominal tenderness	Non specific	CK 20, CDX2 and MUC2,MUC5AC	Low-attenuation Scalloping of the visceral surfaces differentiates pseudomyxoma from other causes of peritnoitis. Typically does not invade visceral organs or spread by lymphatic or hematogenous routes unlike mucinous carcinomatosis	The mucin has high signal intensity on T2-weighted MR images and low signal intensity on T1-weighted images	The echoes within pseudomyxoma peritonei are not mobile Echogenic septations within the gelatinous ascites are frequently observed. Scalloping of the hepatic and splenic margins	Gelatinous ascites in peritoneum and visceral organs,usually right hemidiaphragm, liver.	Diagnostic laparascopy/laparatomy
Peritoneal carcinomatosis without mucinous ascites	Abdominal pain Abdominal distention	Negative	Abdominal distention Abdominal pain	Non specific		Pleural effusion and/or masses in chest Mesenteric or retroperitoneal lymphadenopathy Omental caking, and invasion into parenchymal organs		Echoes are mobile No scallioping of visceral organs.
Peritoneal sarcomatosis	Abdominal pain Abdominal distention Bloating Nausea	Negative	Abdominal mass	Non specific		Heterogeneous bulky masses Hypervascularity with or without Hemoperitoneum Variable presence of ascites.				CT-guided percutaneous tissue biopsy
Lymphomatosis	Abdominal pain Blaoting Swollen lymph node	Fever Night sweat Weight loss	Splenomegaly Lymphadenopathy Abdominal mass Abdominal tenderness	Anemia Pancytopenia Thrombocytopenia Thrombocytosis	CD20, CD79a, CD10	Mostly non-specific Aneurysmal dilatation of the bowel loop with wall thickening may differentiate lymphomatosis from other peritoneal diseases.			Atypical lymphoid cells	Biopsy and histology	Rare manifestation of lymphoma, seen most frequently with non-Hodgkin lymphoma Associated with Herpes virus 8 (HHV-8), which is also associated with Kaposi sarcoma, and Epstein-Barr virus
Tuberclousis Peritonitis	Abdominal pain	Fever Night sweat Weight loss	A positive tuberculin skin test Ascitic fluid will have an elevated white blood cell count with a lymphocytic predominance	Elevated serum CA 125 level Normal serum CA 19-9, and CEA associated with lymphocyte predominant benign ascites		Miliary microabscesses in the liver or spleen lymph node calcification Inflammatory thickening of the terminal ileum and cecum low-attenuation lymphadenopathy			Caseating granuloma with central area of necrotic acellular debris surrounded by histiocytes	Laparoscopy and peritoneal biopsy	Tuberclousis
Endometriosis	Menstrual cramps Painful bowel movements Painful urination during menstrual periods Heavy menstrual periods Pain during or after sex	Negative	Immobile uterine Cervical motion tenderness Fixed and retroverted uterus Tender adnexal mass Tender uterosacral nodularity	Thomsen-Friedenreich (T) antigen (Gal beta1-3GalNAc)					Endometrial-type glands and stroma, as well as hemosiderin-laden macrophages Hemorrhagic, reddish brown or blue nodules or cysts on the peritoneal surfaces	Visual inspection with laparascopy
Sclerosing encapsulating peritonitis	Fever, Weight loss, Loss of appetite Altered bowel movement Episodes of intermittent, partial small bowel obstruction					Diffusely thickened peritoneum and ascites Small bowel may be matted within loculated fluid collections			Dense,opaque peritoneum Progressive collagen formation with an associated chronic inflammatory infiltrate
Gliomatosis Peritonei	Abdominal pain Bloating	Negative		SOX2+/OCT4-/NANOG- immunophenotype					Mature glial tissue in the peritoneal cavity Micronodular growth pattern		Association with solid or immature ovarian teratomas, but it has also been reported to occur in association with ventriculoperitoneal shunts
Osseous metaplasia	Abdominal pain Nausea Vomiting Bloating	Negative				Multiple, high-attenuation, linear-branching structures within the mesentery that extend to the peritoneal surfaces
Splenosis	Abdominal mass	Negative							Lesions are typically multiple, small, reddish-brown nodules that range in size from a few millimeters to 7 cm
Melanosis		Negative							Pigmentation within the dermoid, omentum, and peritoneal cavity Pigment-laden macrophages within the mucosa on PAS staining	Biopsy and histology	Enteric duplication cyst Associated with ovarian cystic teratomas
Inflammatory Pseudotumor	Fever Weight loss Anemia	Negative		Anemia Thrombocytosis Polyclonal hypergammaglobulinemia Stain positive for ALK with immunohistochemistry					Loose myxoid areas with interspersed spindle cells, Variable vascularity Areas of dense collagen, and dense spindled myofibroblasts admixed with inflammatory cells to include plasma cells and lymphocytes.		Chromosomal translocations involving the ALK gene

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