Giant transverse colonic mesenteric mucinous liposarcoma combined with rectal cancer and aortic coarctation: A case report and review of literature

Abstract

BACKGROUND

Liposarcomas (LPSs) are malignant mesenchymal tumors originating from adipocytes. Myxoid LPS (MLPS), a common subtype, predominantly arises in the extremities, retroperitoneum, and deep soft tissues, with a rare occurrence in the gastrointestinal tract. Primary mesenteric LPS is particularly uncommon, especially in the transverse colonic mesentery.

CASE SUMMARY

This report describes the case of a 65-year-old female patient who presented with abdominal distension and was diagnosed with a giant mucinous LPS of the transverse colonic mesentery. Upon admission, the patient underwent a comprehensive evaluation. Contrast-enhanced computed tomography (CT) of the chest and abdomen revealed a large malignant tumor with aortic dissection, while colonoscopy identified rectal cancer. Given the patient's condition and surgical risk, an interventional procedure was first performed to manage the aortic coarctation, followed by tumor resection via laparotomy. Intraoperative and histopathological findings confirmed a giant mucinous LPS originating from the transverse colon mesentery. Postoperatively, the patient underwent chemotherapy and regular follow-up CT. Although the rectal tumor did not recur, the mesenteric tumor showed extensive recurrence, compressing the intestinal lumen and causing mechanical obstruction, which severely threatened the patient's life. A second operation temporarily relieved the obstructive symptoms; however, it had a limited effect on tumor progression. The patient died four months later due to disease progression.

CONCLUSION

While no standardized treatment exists for MLPS co-occurring with multiple diseases, operation remains the mainstay. However, recurrence, metastasis, and poor postoperative prognosis continue to pose serious threats to patient survival.

Key Words: Mucinous liposarcoma; Transverse colonic mesentery; Aortic dissection; Rectal cancer; Intestinal obstruction; Case report

Core Tip: To the best of our knowledge, this is the first reported case of a giant mucinous liposarcomas (LPSs) of the transverse colonic mesentery coexisting with rectal cancer and aortic coarctation. This case contributes valuable clinical insight and highlights the unfavorable outcomes of recurrent and metastatic myxoid LPS.

INTRODUCTION

Liposarcomas (LPSs) are soft-tissue sarcomas originating from adipose tissue. Their clinical presentation ranges from benign localized lesions to aggressive malignant tumors. LPSs are classified into four subtypes: (1) Hyperdifferentiated; (2) Dedifferentiated; (3) Pleomorphic; and (4) Mucinous-like[1-3]. Myxoid LPS (MLPS) accounts for approximately 5% of all soft-tissue sarcomas and 20%–30% of all LPS, with no sex predilection[4,5]. MLPS predominantly arises in the deep soft tissues of the extremities and retroperitoneum, with a high risk of recurrence even after complete resection. It is less common in the abdomen, accounting for approximately 20% of adult human mesenchymal malignancies[6,7]. It has been reported in the mesentery of the small intestine and transverse colon; however, cases involving the mesentery of the transverse colon are sparsely documented in the literature[8]. This report describes an exceptional case of giant mucinous LPS in the transverse colonic mesentery, combined with rectal cancer and aortic entrapment, presenting primarily as an abdominal mass. Intraoperative findings confirmed the tumor’s origin in the transverse colon mesentery. A literature review of related conditions is also included.

CASE PRESENTATION

Chief complaints

A 65-year-old woman presented to our general surgery department with an unexplained abdominal distension.

History of present illness

The patient experienced significant abdominal distension and fatigue but reported no abdominal pain, diarrhea, nausea, vomiting, or difficulty with defecation. There was noticeable weight loss, and the gas output was reduced. These symptoms persisted for over a month without any treatment.

History of past illness

The patient had a history of hypertension.

Personal and family history

There was no family history of malignancy, psychological disorders, or genetic disorders.

Physical examination

Vital signs were stable. Physical examination revealed a distended abdomen with a large, ill-defined, smooth, and minimally mobile mass on palpation. Abdominal percussion elicited a drum sound.

Imaging examinations

An abdominal ultrasound conducted at a local hospital indicated a large solid mass in the abdominal cavity, prompting further examination. Upon admission, the patient underwent contrast-enhanced computed tomography (CT) of the chest and abdomen, revealing a large, inhomogeneous hypoechoic mass with aortic coarctation (Figure 1). An electronic gastroenteroscopy was performed to determine the tumor’s origin. The first attempt failed due to the patient’s abdominal distension and poor physical condition. However, after repeated persuasion, a second attempt was successful, revealing a small elevated lesion 10 cm from the anal verge. A biopsy confirmed rectal cancer (Figure 2). The tumor was small and not detectable on CT. Other examination results indicated multiple comorbidities and poor physical condition.

Figure 1 Preoperative ancillary investigations following hospital admission and ancillary investigations before discharge. A: Contrast-enhanced abdominal computed tomography (CT) showing a large intra-abdominal tumor measuring approximately 25 cm × 20 cm; B: Contrast-enhanced chest CT revealing an eccentric, curvilinear filling defect in the thoracic aorta, accompanied by mild luminal stenosis and rigidity; C: Electronic colonoscopy showing a small protruding lesion in the rectum occupying the lumen for about 1/4 weeks, with central ulceration, and a stiff, brittle texture; D-F: First postoperative thoracic and abdominal CT review; G-I: Second postoperative thoracic and abdominal CT review. Comparative analysis across the three image sets demonstrates the progression and response to treatment.

Figure 2 Histopathological examination. A: Rectal tumor: Moderately differentiated tubular adenocarcinoma of the rectum [hematoxylin and eosin staining (HE): 100 ×]; B: Focal hypercellularity was observed within an otherwise hypocellular tumor embedded in interstitial mucinous stroma of mucinous liposarcoma (HE: 100 ×); C: Spindle-shaped tumor cells of varying sizes with multifocal nuclear atypia of Mucinous liposarcoma (HE: 400 ×).

FINAL DIAGNOSIS

The CT imaging could not determine the tumor’s nature, necessitating further clinical evaluation. The patient was diagnosed with a large abdominal tumor, rectal cancer, and aortic dissection (AD).

TREATMENT

Cases involving abdominal tumors combined with aortic coarctation are rare in the literature and clinical practice. Given the patient's advanced age and multiple comorbidities, particularly aortic coarctation, which poses a high risk of vascular rupture, a multidisciplinary team opted to address the aortic condition primarily.

According to the literature, AD involves tearing the aortic intima, allowing blood to enter the aortic media, and separating the true and false lumens, accounting for approximately 80%–90% of acute aortic syndromes[9]. Based on the Debakey and Stanford classifications, as well as the expanded “non-A and non-B” categorization and time-based staging systems[10,11], this patient was diagnosed with acute, uncomplicated Stanford type B AD. In such cases, optimal medical therapy (OMT), primarily focused on blood pressure control, is the conventional initial treatment. OMT helps reduce the incidences of aneurysmal degeneration, early morbidity, and mortality[12]. However, long-term outcomes reveal a high complication rate, with 20%–40% of patients developing aneurysmal changes and up to 45% eventually requiring surgical intervention[13]. Recent studies support thoracic endovascular aortic repair as superior to medical therapy alone, offering improved aortic wall remodeling and reducing adverse event risks[14]. Considering the patient's condition, surgical risks, and recovery capacity, thoracic aortic stent placement was successfully performed in the interventional suite, followed by abdominal surgery after stabilization (Figure 3).

Figure 3 Surgical images. A: Preoperative model of thoracic aortic stenting; B: Large tumor originating from the transverse colonic mesentery of gross intraoperative specimens; C: Cross-section showing a soft, greyish-white to yellowish mucinous consistency; overall dimensions: 40 cm × 20 cm × 10 cm, uniform across all sections of gross intraoperative specimens.

Abdominal surgery was performed in the operating room. Intraoperatively, a large abdominal mass, measuring approximately 25 cm × 20 cm, was identified, with an irregular shape, incompletely encapsulated by peritoneum, a brittle and friable texture, and coated with a mucoid substance (Figure 3). Further exploration revealed that the tumor originated from the mesentery of the transverse colon. No evidence of distant metastasis was observed in the abdominal cavity or peritoneum. Complete resection of the tumor and the involved colon segment was achieved, followed by anastomosis. The rectal tumor, located above the peritoneal reflection, was small and had not infiltrated the serosa. Segmental resection of the rectum and sigmoid colon was performed to ensure complete removal, followed by anastomosis. Gross examination revealed a 40 cm × 20 cm × 10 cm grayish-yellow mucoid mass on the cross-section (Figure 3). Postoperatively, both resected specimens were sent for pathological analysis, and histological examination by experienced pathologists confirmed a diagnosis of MLPS (Figure 2). The rectal tumor infiltrated the superficial muscularis propria without involving the deeper muscularis layer with negative margins, and no metastasis of the regional lymph nodes was identified (Figure 2). Based on to the tumor-node-metastasis staging criteria, the patient was diagnosed with stage I (pT2N0M0) rectal cancer.

OUTCOME AND FOLLOW-UP

The patient was discharged from the hospital 24 days postoperatively without any immediate complications. Based on oncology consultation, a FOLFOX chemotherapy regimen (consisting of 5-fluorouracil, oxaliplatin, and leucovorin) was initiated, alongside regular follow-up CT scans. However, abdominal CT and positron emission tomography-CT scans conducted 6 months postoperatively revealed multiple irregular low-density areas around the right margin of the liver and the right paracolic region, indicating a recurrence of MLPS. Given disease progression and with the consent of the patient's family, the chemotherapeutic regimen was changed to paclitaxel combined with platinum. However, the patient did not comply with the treatment. One year postoperatively, the tumor gradually enlarged, compressing the intestinal canal and narrowing the lumen, ultimately resulting in a mechanical intestinal obstruction that posed a severe life-threatening risk. A second cesarean section was urgently performed to relieve the obstruction. Due to the presence of multiple tumors, widespread metastasis, and tight adhesion to the abdominal wall, intestines, and surrounding tissues, radical resection was not possible. A substantial portion of the tumor was removed, successfully alleviating the intestinal obstruction. A postoperative CT scan showed a slight reduction in tumor volume compared to the previous scan (Figure 1). The patient survived for four months after the second surgery, with an overall survival of 19 months from the diagnosis.

DISCUSSION

LPS, a malignant tumor derived from adipocytes, includes MLPS as a common subtype. The MLPS is typically found in deep soft tissues of the extremities or retroperitoneum, with rare occurrences in the abdomen[15]. Clinical presentation is usually atypical, and tumors are frequently large at diagnosis[16]. Imaging tests such as CT or magnetic resonance imaging are essential for prompt diagnosis and treatment. Mucinous and non-mucinous tumors can be differentiated through puncture biopsy after tumor confirmation[17]. Moreover, DNA Damage Inducible Transcript 3 immunohistochemistry effectively distinguishes MLPS from other LPS subtypes[18].

Surgical resection is the primary treatment for MLPS. Limb-preserving resection is standard for tumors in the limbs and trunk, depending on the tumor’s location and size. There are no specific surgical guidelines for abdominal MLPS; this approach mirrors that for LPS, involving resection of extensive tissues with ≥ 10 mm margins of adjacent negative tissue[19]. A recent literature review indicates that this approach is applied to all primary mesenteric MLPS (Table 1)[20-38]. Due to the high recurrence rates of MLPS, adjuvant treatments such as postoperative radiotherapy and chemotherapy are used. MLPS is more sensitive to radiotherapy, with preoperative moderate-dose radiotherapy improving resection rates and postoperative radiotherapy reducing recurrence risk[39,40]. Chemotherapy’s effectiveness in MLPS, particularly in advanced or metastatic cases, is limited. Anthracycline-based regimens, such as “adriamycin + isocyclophosphamide”, are commonly used, though results are frequently unsatisfactory[41]. Recently, second-line chemotherapeutic agents such as trabectedin, pazopanib, and eribulin have shown some efficacy[42]. Trabectedin mechanistically inhibits the transcription of the oncogenic fusion protein fused in sarcoma-DNA damage-inducible transcript and is more radiosensitive, making it an effective combination with radiation for treating MLPS[43,44]. Moreover, the potential of immunotherapy and targeted therapies in MLPS treatment has garnered considerable attention. For advanced patients with unresectable tumors, immunotherapies, such as natural killer cell therapy or tumor microenvironment modulation, could provide promising new treatment options[45]. Following MLPS resection, circulating tumor DNA can be a non-invasive and cost-effective method for monitoring tumor activity, enabling the early detection and treatment of recurrence[46]. However, these approaches remain in the research phase and require further validation for clinical application.

Table 1 Clinical and histopathologic features, follow-up, and clinical outcomes of patients with mesenteric liposarcoma published in recent 10 years.

Ref.	Age/sex	Size (cm)	Wide excision	Pathological type	Location	Adjuvant therapy	Local recurrence/metastases	Follow-up time (months)
Rowe et al[20]	76/F	15 × 9 × 15	Yes	D	Large intestine mesentery	No	No	6
Ahire et al[21]	42/M	20 × 15	Yes	D	Jejunum mesentery	Chemotherapy	No	6
Mokfi et al[22]	69/F	29 × 25 × 12	Yes	Myx	Small bowel mesentery	No	No	6
Park et al[23]	47/M	25.5 × 19.0 × 12.5	Yes	D	Ascending colon mesentery	Chemotherapy	No	21
Dhakal et al[24]	56/M	12 × 10 × 7	Yes	D	Small bowel mesentery	No	N/A	N/A
Poillucci et al[25]	43/M	12 × 12 × 9	Yes	W	Small bowel mesentery	No	No	N/A
	60/M	Diameter 9, 10 and 7.5	Yes	W	Small bowel mesentery	No	No	N/A
Kuroda et al[26]	67/F	11 × 10 × 9	Yes	D	Transverse colon mesentery	Chemotherapy	Dead (18 months)	18
Khanduri et al[27]	55/M	6 × 7 × 8	Yes	D	Jejunum mesentery	Chemotherapy	No	2
Mori et al[28]	71/M	Diameter is 3.10	Yes	D	Small bowel mesentery	Chemotherapy	Recurrence (17days)	6
Innes et al[29]	65/M	8 × 7.5 × 3	Yes	D	Cecum mesentery	No	Recurrence (2.5 months), dead (3 months)	3
Kim et al[30]	60/M	25 × 20	Yes	Myx	Small bowel mesentery	Chemotherapy	No	6
Paasch et al[31]	35/F	14.6 × 10.1 × 12.4	Yes	Myx	Small bowel mesentery	Chemotherapy	Dead (years)	3 (years)
Zhang et al[32]	65/M	6 × 3 × 3	Yes	D	Cecum mesentery	No	No	6
Jung et al[33]	62/M	N/A	Yes	W	Small bowel mesentery	Chemotherapy	No	2 (years)
Rosato et al[34]	55/M	5 × 5 × 6	Yes	W	Small bowel mesentery	No	No	144
Matsuo et al[35]	70/M	N/A	Yes	D	Small bowel mesentery	No	No	5 (years)
Vats et al[36]	36/F	25 × 20 × 15	Yes	D	Jejunum mesentery	Chemotherapy	No	6
Choi et al[37]	73/M	28 × 26 × 12	Yes	Mix-type liposarcoma	Small bowel mesentery	No	No	33
Shen et al[38]	49/F	26 × 18 × 6	Yes	Myx	Sigmoid mesentery	No	No	17

D: Dedifferentiated liposarcoma; F: Female; M: Male; Myx: Myxoid liposarcoma; N/A: Not available; W: Well-differentiated liposarcoma.

This case highlights the rarity of primary mesenteric MPLS, primarily when originating from the transverse colonic mesentery, with few cases reported in the literature. Furthermore, aortic coarctation adds complexity and risk to the condition. The small size of rectal tumors, frequently undetectable on CT scans, was incidentally discovered during electronic colonoscopy, enabling timely diagnosis and treatment. Given the patient's advanced age and frailty, delayed diagnosis of rectal cancer could have necessitated a second surgery, significantly elevating the risk of complications. Therefore, we promptly adjusted the postoperative surveillance strategy to monitor both primary tumors. Although the initial surgery was successful, the tumor recurred rapidly, leading to malignant intestinal obstruction—the ultimate cause of death. Currently, clinical data and basic research on primary mesenteric MLPS remain scarce. More case reports and mechanistic studies are urgently needed to optimize individualized treatment strategies.

CONCLUSION

The diagnosis of primary mesenteric MLPS is frequently delayed, due to nonspecific clinical presentations, and surgical resection remains the only potentially curative treatment option. Comprehensive ancillary investigations and timely operation are essential for detecting surgically resectable lesions and improving prognosis. Comprehensive imaging and electronic gastroenteroscopy are recommended for all patients with abdominal masses to minimize the risk of missed diagnoses and maximize the chances of long-term disease-free survival. Future research should focus on optimizing comprehensive treatment strategies, implementing a multidisciplinary approach, and advancing early diagnosis and individualized treatment to improve clinical outcomes and prolong survival. There is a pressing need to refine clinical guidelines.

ACKNOWLEDGEMENTS

We sincerely appreciate the patients’ families for their cooperation with information acquisition, treatment, and follow-up.