High-volume mucinous ascites secondary to low-grade mucinous neoplasm arising in a mature cystic teratoma: a case report

Introduction

Background

Pseudomyxoma peritonei (PMP) is a rare and complex clinical syndrome characterized by the accumulation of mucinous material within the peritoneal cavity, typically associated with mucin-producing epithelial neoplasms, and often requires multimodal evaluation for accurate diagnosis (1,2). Historically, PMP was initially described in association with ovarian tumors. However, advances in pathologic classification and clinicopathologic correlation have clarified that the vast majority of cases originate from appendiceal mucinous neoplasms, most often of low histologic grade (3). Appendiceal tumors remain the most common and well-established source of PMP, though mucinous neoplasms arising from other gastrointestinal sites have also been described. Importantly, PMP of appendiceal origin can clinically and radiographically mimic ovarian malignancy, often presenting with pelvic masses, mucinous ascites, and overlapping tumor marker profiles, which may complicate initial diagnostic evaluation (4). Although uncommon, ovarian sources of PMP are clinically relevant and account for a small minority of cases, with estimates suggesting approximately 3% of PMP arises from ovarian primaries (5). Differentiating ovarian-origin disease from appendiceal metastases remains challenging, as these tumors frequently demonstrate overlapping radiologic, morphologic, and immunohistochemical features (6). Recognition of ovarian-origin PMP is therefore essential to ensure accurate diagnosis and appropriate multidisciplinary management.

Rationale and knowledge gap

Among ovarian causes of PMP, mucinous neoplasms arising within mature cystic teratomas represent an exceptionally rare subset. Systematic reviews of the literature have identified malignant transformation of mature ovarian teratomas as a recognized but infrequent ovarian origin of PMP, accounting for only a small fraction of reported cases (5). Diagnosis of these tumors is particularly challenging due to overlapping histologic and immunophenotypic features with appendiceal mucinous neoplasms, as many teratoma-associated mucinous tumors demonstrate gastrointestinal-type differentiation that can obscure determination of the primary site and complicate accurate classification. Recent case series have emphasized that PMP arising from mature cystic teratomas closely mimics appendiceal disease on immunohistochemistry, further complicating accurate classification (7). In addition to histopathologic overlap, imaging findings may also be insufficient to reliably distinguish primary ovarian tumors from metastatic appendiceal disease, particularly when both present with multilocular cystic masses and peritoneal mucin (8). As a result, determination of tumor origin often requires integration of clinical, radiologic, and pathologic data. Although recent case series have expanded recognition of this entity, the overall number of reported cases remains limited, and long-term outcomes, optimal management strategies, and prognostic factors are not well defined (5-7,9-14). This persistent diagnostic ambiguity represents a key gap in the literature. Recent literature continues to emphasize that PMP remains a diagnostically and therapeutically challenging entity with an evolving understanding of its pathogenesis and optimal management strategies (15).

This case is notable for the exceptionally high volume of mucinous intraperitoneal material, the presence of a gastrointestinal-type immunophenotype in the absence of appendiceal pathology, and the resulting diagnostic complexity in determining the primary site of disease. By highlighting these features, this report aims to further clarify the clinical and pathologic spectrum of this rare entity.

Objective

The objective of this report is to describe a rare case of high-volume mucinous ascites secondary to a low-grade mucinous neoplasm arising within a mature ovarian cystic teratoma. By contributing to the limited existing literature, this case aims to increase clinical awareness of this uncommon diagnosis and highlight the importance of comprehensive clinicopathologic evaluation in patients presenting with mucinous peritoneal disease. We present this article in accordance with the CARE reporting checklist (available at https://acr.amegroups.com/article/view/10.21037/acr-2026-0025/rc) (16).

Case presentation

We report the case of a 30-year-old nulliparous woman who first noticed increased abdominal pain, fullness, and discomfort. Her symptoms progressively worsened, and she presented to a local emergency department for care. She had a past medical history of hypertension, type 2 diabetes mellitus, and obesity, as well as a family history of breast cancer in a second-degree relative. Computed tomography noted a complex, cystic, solid, partially calcified, and partially macroscopic fat-containing mass centered in the abdomen, with large-volume intermediate-density mucinous ascites consistent with mucinous material (Figure 1). The appendix was visualized and demonstrated a normal appearance without evidence of wall thickening, luminal dilation, or periappendiceal inflammation. Aspiration of the presumed mucinous ascites yielded 80 mL of mucinous fluid. She underwent colonoscopy and upper endoscopy, which were unremarkable. Tumor markers were notable for carcinoembryonic antigen (CEA) of 717.1 ng/mL, cancer antigen 125 (CA125) of 18.0 units/mL, and cancer antigen 19-9 (CA19-9) of 225.2 units/mL. She was referred to a higher acuity center for further management. The patient’s clinical course is summarized in Table 1.

Figure 1 Cross-sectional imaging demonstrating extensive mucinous ascites and ovarian origin of disease. (A) Axial CT: large volume ascites with central area of macroscopic fat (arrow i) and calcification (arrow ii). (B) Axial CT: there is a hyperattenuating soft tissue component along the right peripheral margin of the lesion (arrow iii). The mass originates from the left ovary after following the gonadal vessels (arrow iv). (C) Sagittal CT: craniocaudal extent of the mass (white line) measured slightly over 30 cm. Areas of macroscopic fat (single arrows), and calcification (double arrow), are noted within the mass. (D) Coronal CT: note the normal, air-filled, non-dilated appendix (arrow v). Additional note is made of a right ovarian teratoma containing calcification and macroscopic fat (arrow vi). CT, computed tomography.

The initial surgical consultation was conducted via telehealth due to the distance between her residence and the hospital. Surgical exploration was recommended to establish a diagnosis, and she agreed to proceed. Surgery was performed on December 3, 2025, with a total operative time of approximately 2 hours. On the day of surgery, her physical exam was notable for a tense and significantly distended abdomen, causing moderate tachypnea and increased work of breathing. Her vital signs were notable for a heart rate of 117 beats per minute and an oxygen saturation of 99% on room air, with a respiratory rate of 24 breaths per minute, consistent with physiologic impact from the mass effect of the intra-abdominal process. Her body mass index was 54.8 kg/m², consistent with class III obesity. The remainder of her examination was within normal limits.

Intraoperative findings demonstrated a large volume of mucinous intraperitoneal fluid and a markedly enlarged left ovary exceeding 20 cm in diameter (Figure 2). The appendix appeared grossly normal without evidence of enlargement, perforation, or mucinous involvement. Intraoperative frozen section analysis was not performed, and surgical decision-making was based on gross intraoperative findings and clinical suspicion, with definitive diagnosis established on final histopathologic evaluation. There was peritoneal nodularity throughout the abdomen, including in the right upper quadrant. We also noted some nodular lesions in the omentum and an enlarged right ovary, approximately 8–10 cm. We proceeded to evacuate approximately 22 L of mucinous fluid from the abdomen, which likely contributed to the patient’s preoperative abdominal distension and respiratory compromise, and completed an appendectomy, left oophorectomy, partial omentectomy, and multiple peritoneal biopsies (Figure 3). Histopathologic evaluation of the biopsied peritoneal nodules demonstrated predominantly dense acellular mucin with associated inflammatory changes, while abdominal mucin specimens contained focal low-grade mucinous epithelium consistent with mucinous carcinoma peritonei.

Figure 2 Intraoperative photograph demonstrating severe abdominal distension secondary to high-volume mucinous ascites (A) before and (B) after surgical decompression. The tense abdominal wall reflects the significant mass effect caused by intraperitoneal mucin accumulation, which contributed to the patient’s presenting symptoms and necessitated operative intervention.

Figure 3 Gross intraoperative specimens demonstrating ovarian mass and evacuated mucinous ascites. Intraoperative photographs demonstrating (A) the resected ovarian mass with associated mucinous material, grossly consistent with a mucin-producing neoplasm arising within a mature cystic teratoma, and (B) a basin containing thick, gelatinous mucinous ascites evacuated from the peritoneal cavity. The extensive volume and viscosity of the mucinous fluid are characteristic of mucinous peritoneal disease and contributed to the patient’s presenting symptoms.

The estimated blood loss was approximately 100 mL. The patient underwent general anesthesia with endotracheal intubation and was successfully extubated in the operating room. No intraoperative complications were encountered. Given the high-volume evacuation of mucinous intraperitoneal material, careful hemodynamic monitoring was maintained throughout the procedure, and the patient remained stable without evidence of hemodynamic instability or respiratory compromise. After surgery, she progressed well. She was discharged on postoperative day three on a regular diet and oral pain medication without issue.

Pathology was consistent with low-grade mucinous epithelial neoplasm associated with mature cystic teratoma, as well as extensive pools of extravasated acellular mucin with associated granulomatous reaction involving the serosa of the fallopian tube and ovarian surface, extensive pools of acellular mucin, and associated low-grade mucinous carcinoma peritonei and dystrophic calcifications (Figure 4). The tumor involving the left ovary demonstrated a mucinous histomorphology with goblet cells, low nuclear grade, and extensive pools of extravasated mucin. The neoplastic cells display an appendiceal-type immunophenotype, with diffuse expression of cytokeratin 20, CDX2, and SATB2, focal expression of cytokeratin 7, and no expression of PAX8, estrogen receptor (ER), or progesterone receptor (PR). The appendix was entirely submitted for histologic examination and demonstrated no evidence of primary appendiceal neoplasm or dysplasia. Histologic evaluation showed reactive changes, including lymphoid hyperplasia and fibrous obliteration of the appendiceal tip, with acellular mucin involving the serosa but no mucosal involvement or neoplastic epithelial proliferation.

Figure 4 Histopathologic features of a mucinous neoplasm arising within a mature cystic teratoma. (A) Mature teratoma with skin (lower right) and cysts lined by mucinous epithelium (upper and lower left) (H&E, 2×). (B) Mucinous neoplasm composed of columnar proliferative epithelium without significant cytologic atypia (H&E, 20×). H&E, hematoxylin and eosin.

Telehealth follow-up was completed 21 days postoperatively, at which time the patient was recovering well, with improved symptoms including decreased abdominal discomfort and improved oral intake, and no concerns regarding her incision. There were no patient-reported symptoms suggestive of early recurrence or persistent ascites at that time. She was scheduled for repeat cross-sectional imaging approximately 6 weeks after surgery to reassess the volume of retained mucin; however, this was unable to be obtained given her lack of insurance coverage and inability to pay for. Long-term surveillance with serial cross-sectional imaging and clinical follow-up was planned every 3 months for 2 years given the risk of disease recurrence.

All procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee(s) and with the Declaration of Helsinki and its subsequent amendments. Written informed consent was obtained from the patient for the publication of this case report and accompanying images. A copy of the written consent is available for review by the editorial office of this journal.

Discussion

Key findings

Our case describes a rare presentation of PMP arising from a low-grade mucinous neoplasm within a mature ovarian cystic teratoma. The case is notable for the extremely large volume of intraperitoneal mucin, the ovarian origin of disease in a young patient, and the absence of appendiceal pathology, all of which contributed to diagnostic complexity. While PMP most commonly originates from appendiceal mucinous neoplasms, ovarian sources, particularly those arising from mature cystic teratomas, have been increasingly recognized as rare but clinically important alternative primary sources (7,11). The diagnostic challenge in this case was further compounded by the gastrointestinal-type immunophenotype of the tumor, which closely mimics appendiceal disease and can obscure determination of the true primary site. Ultimately, pathologic evaluation demonstrating low-grade mucinous neoplasia arising within a teratoma, combined with a grossly and microscopically normal appendix, supported an ovarian origin, in keeping with prior reports (10,11). A key diagnostic consideration in this case was exclusion of an occult gastrointestinal primary, particularly given the tumor’s SATB2 and CDX2 positivity, which is commonly associated with appendiceal or colorectal origin. However, immunohistochemistry alone is insufficient to determine the primary site in these cases, as there is known overlap in marker expression between primary ovarian mucinous tumors and metastatic gastrointestinal neoplasms (6). While SATB2 is highly sensitive for appendiceal and colorectal tumors, its expression has also been reported in ovarian mucinous neoplasms, particularly those arising in association with mature cystic teratomas, limiting its specificity in this context (17,18). In this patient, a comprehensive gastrointestinal evaluation, including colonoscopy and upper endoscopy, was unremarkable. Cross-sectional imaging demonstrated a normal appendix without evidence of a primary lesion, and intraoperative as well as histopathologic evaluation confirmed the absence of appendiceal neoplasia. These findings support an ovarian origin of disease arising within a mature cystic teratoma despite the gastrointestinal-type immunophenotype.

Strengths and limitations

A major strength of this case is the multidisciplinary approach to diagnosis and management, incorporating radiologic imaging, surgical findings, and detailed histopathologic and immunohistochemical analysis. Comprehensive immunohistochemistry was particularly valuable in characterizing the tumor’s gastrointestinal-type differentiation and excluding a non-ovarian primary source. Additionally, this case contributes to the limited literature on mucinous neoplasms arising within ovarian mature cystic teratomas, particularly those presenting with large-volume intraperitoneal mucin. However, several limitations should also be acknowledged. As a single case report, findings are inherently not generalizable. Follow-up is currently short-term, and long-term outcomes such as recurrence or progression remain unknown. PMP is associated with a risk of delayed recurrence, necessitating prolonged surveillance with serial cross-sectional imaging and clinical evaluation. In this case, structured long-term surveillance is planned, including cross-sectional imaging at approximately 6 weeks postoperatively, followed by imaging at 3–6-month intervals during the first year and annually thereafter, in conjunction with clinical evaluation and tumor marker assessment. Repeat imaging will be used to assess the burden of residual mucinous disease and guide consideration of additional cytoreductive surgery (CRS) based on interval progression and clinical status. However, access to imaging has been constrained by the patient’s insurance status, which may impact adherence to standard surveillance protocols. An additional limitation is the absence of molecular testing, such as KRAS or GNAS mutation analysis, which has been shown to provide complementary diagnostic insight and may assist in distinguishing primary from metastatic disease in cases with overlapping clinicopathologic features (19-21). Finally, given the rarity of this entity, standardized management guidelines are lacking, and treatment decisions are predominantly guided by available case series and expert consensus rather than high-level evidence.

Comparison with similar research

Prior studies have demonstrated that PMP arising from ovarian mature cystic teratomas is an exceptionally rare entity. In a recent case series and literature review, Ha et al. described six patients with PMP originating from mature ovarian teratomas, noting that these tumors closely resemble appendiceal neoplasms on immunohistochemistry and that most patients have favorable outcomes following CRS with close surveillance (7). Other case reports have described similar presentations with large-volume intraperitoneal mucin and gastrointestinal-type immunophenotypes, though management strategies have varied. Ponzini et al. reported a case of PMP arising from malignant transformation of an ovarian teratoma that was treated with CRS and hyperthermic intraperitoneal chemotherapy (HIPEC), highlighting a more aggressive approach in advanced disease (22). Similar behavior has been described in rare extragonadal teratomas. Mckenney and Longacre reported a sacrococcygeal mature teratoma associated with an intestinal-type mucinous epithelial neoplasm that later recurred as disseminated low-grade mucinous peritoneal disease, supporting the concept that gastrointestinal-type differentiation within teratomas can give rise to PMP regardless of anatomic location (23). From an imaging perspective, Tanaka et al. demonstrated that mucinous tumors arising from ovarian teratomas can closely mimic ovarian metastases from appendiceal tumors, though features such as unilateral disease, intratumoral fat, and a relatively normal appendix may suggest a teratoma-associated origin (8). These studies reinforce both the rarity of this diagnosis and the ongoing challenges in distinguishing ovarian primary disease from secondary gastrointestinal involvement. Previously reported cases of PMP arising from mature cystic teratomas are summarized in Table 2, highlighting variability in clinical presentation, immunohistochemical profiles, and management strategies. Compared to previously reported cases, our patient demonstrated an unusually high volume of mucinous ascites, exceeding that described in most prior reports.

Explanations of findings

Mucinous tumors arising within mature cystic teratomas are thought to originate from teratomatous gastrointestinal-type epithelium, which explains their morphologic and immunohistochemical similarities to appendiceal mucinous neoplasms (9,10). Molecular studies have demonstrated that these tumors may share key genetic alterations with appendiceal and PMP-associated neoplasms, including mutations in KRAS and GNAS, supporting a common molecular pathway underlying mucinous differentiation (19,21). GNAS mutations in particular have been shown to play a role in mucin production through upregulation of mucin-related gene expression, while co-occurring KRAS mutations contribute to tumor proliferation and progression (24,25). These shared molecular features provide a biologic basis for the overlapping morphologic and immunophenotypic characteristics observed in teratoma-associated mucinous tumors and further complicate the determination of tumor origin. Additionally, studies utilizing next-generation sequencing have demonstrated concordant KRAS and GNAS mutations across appendiceal and ovarian tumor sites in challenging cases, highlighting the role of molecular profiling in distinguishing primary from metastatic disease when conventional methods are inconclusive (20).

The classification of these tumors is currently controversial. The 2020 WHO classification suggests using a harmonized terminology with mucinous tumors of the appendix. However, some studies have recently postulated that the origin from gastrointestinal-type elements within the teratoma is closer to pancreatic mucinous neoplasms, since the latter share ovarian-type stroma, and thus support their origin in the upper gastrointestinal tract (26). Further study, including larger case series, is needed to better define their biologic behavior and clinical implications. The extensive mucinous intraperitoneal material observed in this patient is likely the result of rupture or leakage from the ovarian neoplasm, leading to dissemination of acellular mucin and low-grade epithelial elements. The presence of a grossly and histologically normal appendix in this and other reported cases supports the notion that, although uncommon, teratoma-associated mucinous tumors may represent a true ovarian primary source of PMP. This emphasizes the importance of careful gross and microscopic evaluation of the appendix, as well as integration of clinical, surgical, and pathologic findings to accurately determine tumor origin (11,27).

Implications and actions needed

This case highlights the importance of maintaining clinical awareness of ovarian sources of PMP, particularly in patients with mucinous intraperitoneal fluid and normal gastrointestinal evaluation. Recognition of this rare entity has meaningful implications for diagnostic workup, surgical planning, and referral to specialized centers that have experience managing peritoneal surface malignancies. Given the limited number of reported cases and absence of standardized treatment recommendations, continued case reporting and accumulation of long-term outcome data are essential. Increased awareness among clinicians and pathologists may help reduce diagnostic delays and improve patient care through earlier identification and appropriate multidisciplinary management (5,7). An additional important consideration in this case is the patient’s young age and nulliparous status, which introduces complexity in balancing oncologic management with fertility preservation. CRS with or without HIPEC is often considered in the management of PMP and remains a central component of treatment strategies, with some studies suggesting improved survival when combined with complete cytoreduction (28,29). In selected patients, this approach has also been associated with favorable long-term outcomes, including in cases of ovarian-origin disease (30). However, these interventions routinely involve resection of reproductive organs and can adversely affect ovarian reserve and reproductive potential (31,32). Although successful pregnancies following CRS and HIPEC have been reported, these remain relatively uncommon, and fertility outcomes are not well defined (33). Alternative or staged approaches may be considered in select patients with low-grade disease who desire fertility preservation, though these must be weighed carefully against the risk of disease progression (34). Given the rarity of this entity, institutional experience is limited, and fertility-sparing decisions should be individualized based on disease burden, histologic grade, extent of peritoneal involvement, and patient goals. These considerations underscore the importance of multidisciplinary decision-making and early fertility counseling, as patient-reported experiences suggest that fertility discussions are not consistently addressed in this population (35).

Conclusions

PMP arising from a low-grade mucinous neoplasm within a mature ovarian cystic teratoma is a rare but clinically significant entity that can closely mimic appendiceal mucinous neoplasms. This case adds to the limited literature reporting ovarian mature cystic teratomas as a potential primary source of mucinous peritoneal disease and highlights the diagnostic challenges posed by overlapping morphological and immunohistochemical features. Increased clinical and pathologic awareness of this uncommon diagnosis is important to facilitate accurate identification, particularly through a multidisciplinary approach and consideration of an ovarian teratoma-associated origin with gastrointestinal-type immunophenotypes, and to support informed clinical decision-making. For surgeons encountering high-volume mucinous intraperitoneal fluid without a clear appendiceal source, careful evaluation of the ovaries should be considered as part of the intraoperative assessment.

Acknowledgments

None.

Footnote

Reporting Checklist: The authors have completed the CARE reporting checklist. Available at https://acr.amegroups.com/article/view/10.21037/acr-2026-0025/rc

Peer Review File: Available at https://acr.amegroups.com/article/view/10.21037/acr-2026-0025/prf

Funding: None.

Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://acr.amegroups.com/article/view/10.21037/acr-2026-0025/coif). M.D.T. received grants for clinical trial support from Karyopharm, GSK, and Exelixis (to institution); received payment or honoraria from Intuitive 2024 and Onc Live 2025 (to self); and served on board on Caris Life Sciences—consulting. The other authors have no conflicts of interest to declare.

Ethical Statement: The authors are accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. All procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee(s) and with the Declaration of Helsinki and its subsequent amendments. Written informed consent was obtained from the patient for the publication of this case report and accompanying images. A copy of the written consent is available for review by the editorial office of this journal.

Open Access Statement: This is an Open Access article distributed in accordance with the Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International License (CC BY-NC-ND 4.0), which permits the non-commercial replication and distribution of the article with the strict proviso that no changes or edits are made and the original work is properly cited (including links to both the formal publication through the relevant DOI and the license). See: https://creativecommons.org/licenses/by-nc-nd/4.0/.

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