Giant myxoid retroperitoneal liposarcoma in a 41-year-old patient: A case report

Abstract

Introduction

Retroperitoneal Liposarcomas (RL) are a rare form of malignant tumors, they encompass just 5 % ot these and mainly present in female adults between 50 and 60 years of age. They can be divided into 4 morphological subtypes: 1) well-differentiated, 2) undifferentiated, 3) myxoid, and 4) pleomorphic.

Case presentation

41-year-old male who present to the outpatient consult of oncological surgery after being diagnosed via imaging with a giant retroperitoneal liposarcoma. The patient reported unsuccessful weight loss attempts and subsequently noticed an increase in abdominal size as well as the development of ulcers in the lower limbs. He underwent radical resection, which included a right nephrectomy, as the primary treatment.

Discussion

Giant retroperitoneal liposarcomas are defined as those 30 cm in diameter or more or 20 kg or more in weight are considered “giants” and are extremely rare. The only treatment involves resection of the tumoration and those adjacent involved organs, and sometimes, uninvolved ones. Due to the high risk of recurrence, adjuvant therapy may be provided as well as imaging follow up.

Conclusion

RL are a complicated entity to manage. They tend to present high rates of recurrence due to the difficulty of performing a full resection without positive margins. These patients should be thoroughly studied before surgery and appropriate follow-up should be provided.

Highlights

• •Retroperitoneal Liposarcomas represent a rare form of neoplasia. The giant form represents an smaller fraction of these.
• •They present until they are in an advanced stage and usual manifest with compression symptoms
• •They represent a surgical challenge because of its tendency to invade; not resecting adyacent organs is almost impossible.
• •They require constant follow-up to discard any recurrence

1. Introduction

Soft tissue sarcomas encompass a heterogeneous group of tumors, comprising 1 % of malignant tumors in adults. Among these, retroperitoneal sarcomas account for 20 % of soft tissue sarcomas [1], with the myxoid subtype comprising 5 % of adult cases. Retroperitoneal liposarcomas originate from primitive mesenchymal cells and undergo adipocyte differentiation. The peak incidence of these tumors occurs between the ages of 50 and 60. They are categorized into 4 morphological subtypes: 1) well-differentiated, 2) undifferentiated, 3) myxoid, and 4) pleomorphic [1]. Retroperitoneal sarcomas typically remain asymptomatic until they grow large enough to compress adjacent organs, often leading to misdiagnosis due to the absence of noticeable symptoms. Current diagnostic procedures rely heavily on anatomopathological findings, with techniques such as tomography and magnetic resonance imaging being responsible for the majority of pre-surgical diagnose. While anatomopathological analysis remains the gold standard, the presence of macroscopic fat in imaging studies strongly suggests the presence of myxoid liposarcomas [2]. The size and volume of liposarcomas are highly variable, with “giant” tumors exceeding 30 cm in diameter or weighing over 20 kg being exceptionally rare [3]. Upon diagnosis, surgical intervention is the recommend primary treatment for retroperitoneal liposarcomas. The extent of resection remains controversial, as it is conventional to completely resect adjacent organs, which contrasts with the proposal of partial resection of uninvolved adjacent organs [2]. The purpose of these report is discuss the management of one of the rare presentation of retroperitoneal liposarcoma as well as the outcome of such.

2. Case report

We present a case of a 41-year-old patient with a medical history of Type 2 Diabetes and Arterial Hypertension. The patient's condition began six months before consultation after initiating non-surgical bariatric treatment for weight loss, which did not yield desired results. Instead the patient reported a gradual increase in abdominal circumference, presence of venous ulcers in both lower limbs, and occasional constipation. Upon evaluation, an abdominal ultrasound was performed, revealing an abdominal mass with a volume of 13,754 cc. The patient was subsequently referred to oncological surgery outpatient clinic, where contrast-enhanced abdominal computed tomography was conducted. The imaging study identified a retroperitoneal tumor with partially defined borders, measuring 27.6 × 26.8 × 26.2 cm, causing displacement of the liver, right kidney, small intestine, and colon, without evidence of lymphadenopathy. Physical examination revealed a firm, non-depressible, non-painful, globose abdomen with abundant adipose tissue (Fig. 1). The patient exhibited 3+ godet edema in the lower extremities and ulcerous lesions in the bilateral pretibial region. Six months after the initial diagnosis, the patient underwent surgery to resect the heterogeneous tumor located in the retroperitoneum, which displaced intra-abdominal organs; The tumor respected the Inferior Vena Cava and the Caudate lobe of the liver. The mass was dissected from adhesions to the omentum, posteriorly identifying the gonadal vessels which were dissected and preserved. A subsequent right nephrectomy with its accompanying adrenal gland was performed as the kidney was enveloped within the tumor capsule (Fig. 2). The patient was admitted to the intensive care unit and was ultimately discharged without complications 72 h post-surgery. Definitive histopathological diagnosis reported a high-grade myxoid liposarcoma (Fig. 3) with a tumor size of 33 × 31 × 29 cm and a weight of 15.5 kg. The diagnosis further indicated that necrosis was present in less than 50 % of the tissue and that the resection margins were in contact with the neoplasm. Finally, angiolymphatic invasion was observed, and the kidney exhibited hydropic degeneration of the tubular epithelium and vascular congestion.

Fig. 1.

Image of the patient with an abdominal mass together with a CT scan showing renal involvement.

Fig. 2.

Image of retroperitoneal sarcoma during resection and specimen.

Fig. 3.

Image shows 3 types of components in this photo. From right to left on the first image, cells with lipomatoid characteristics can be seen, followed by sarcomatous cells and a mixoid component. On the second image, mixoid component.

3. Discussion

This case can be classified as a giant liposarcoma, as the tumor exceeded 30 cm in diameter, despite weighing less than 20 kg. In cases of kidney involvement, it is advisable to perform a nephrectomy, which is associated with a 5-year survival of 60–90 % in tumors with myxoid characteristics [4]. Preoperative biopsy was not performed, as evidence suggests, when a liposarcoma is suspected on the retroperitoneum they are more likely to be one that requieres surgical intervention [5]. The clinical presentation in our case was atypical, as these tumors often remain asymptomatic. However, it is common for patients to present with compression-related symptoms affecting urological, gastrointestinal, neurological, or vascular functions [6]. In our patient's case, symptoms included failure to lose weight and the development of venous ulcers due to compression of the vena cava by the tumor. The preferred treatment approach is the complete resection of the tumor and invaded organs, ensuring removal of all tumor remnants. This surgical procedure is challenging due to the extensive tumor size and involvement of adjacent structures. Given the high recurrence rate, especially in cases with positive margins on biopsy and the potential correlation between tumor size and recurrence rate as it is currently discussed [7], it is recommended to perform radiotherapy and/or adjuvant chemotherapy to reduce the likelihood of recurrence. Follow-up imaging with tomography should be performed every three months for the initial two years, every six months for the subsequent two to five years, and then annually to monitor for any signs of recurrence [8]. Appropriate follow-up should always be performed, as was discussed before. RL can present within a different range of subtypes, management should be tailored to each particular case, but as with our patient, surgical treatment is the angular stone of it.

4. Conclusion

Giant retroperitoneal liposarcomas pose significant surgical challenges due to their extensive size and involvement of neighboring structures, as experienced in this case, which required right nephrectomy. Prior to surgery, comprehensive evaluation of the affected area and consideration of resecting adjacent structures should be undertaken. Given the high recurrence rate associated with this tumor type, adjuvant therapy should be considered in order to improve survival. Notably, the presence of clear surgical margins and tumor grade serve as important prognostic factors in determining patient outcomes.

This work has been reported in line with the SCARE criteria [9].

Ethical approval

There was no need for ethical approval from our institution as no form of experimentation was performed. Only written consent was required which was obtained at Hospital Regional Monterrey- Instituto de Seguridad y Servicios Sociales de los Trabajadores del Estado.

Funding

No Funding was received.

CRediT authorship contribution statement

César Eduardo Díaz de León-Romero, MD, Data collection, data analysis, writing the paper.

Miguel Jiménez-Yarza, MD, Data collection, data analysis, writing the paper.

Carlos Eduardo Pérez-Tristán, MD, Data collection, data analysis.

Luis Ernesto Jiménez-Yarza, MD, Data collection, data analysis.

Rodolfo Lucano Valdes-Ramos, MD, Data collection, data analysis.

José Daniel Ortiz-Cisneros, MD, Data collection, data analysis, final approval.

Guarantor

César Eduardo Díaz de León Romero.

Miguel Jiménez Yarza.

Registration of research studies

N/A.

Declaration of competing interest

There are no existing conflicts of interest.