Large Retroperitoneal Tumor Research Articles

Hyper-realistic rendering-assisted laparoscopic adrenalectomy for giant adrenal tumors: a pilot study.

This study aimed to explore the application value of hyperrealistic rendering (HRR) in laparoscopic giant adrenal tumor resection. We retrospectively analyzed 25 patients with giant adrenal tumors from January 2021 to January 2024, with a median age of 56 (40.5, 58.5) years and a tumor median diameter of 7.20 (6.80, 8.50) cm. All patients underwent preoperative medical HRR based on enhanced computed tomography, followed by laparoscopic adrenal tumor resection. HRR was used to initially determine the nature of the tumor and develop a detailed surgical plan, which was completed in 25 patients preoperatively. All 24 cases of tumors were located in the adrenal gland, 1 case was located in the retroperitoneum, and 13 and 12 cases were on the left and right side, respectively. Preoperative HRR 3D imaging was consistent with the intraoperative situation, and 25 cases had successful surgeries. The median operation time was 165 (120.0, 250.0) min, and median bleeding and blood transfusion volume were 200 (150.0, 450.0) and 200.0 (150.0, 450.0) mL, respectively. There were no collateral injuries to important organs and major vessels and no cases of conversion to open surgery. For large retroperitoneal adrenal tumors, HRR for three-dimensional (3D) reconstruction imaging enables the operator to fully understand the relationship between the tumor and surrounding organs and blood vessels preoperatively, which can reduce intraoperative bleeding and collateral injuries, improve the success rate of laparoscopic resection, and safety of the operation.

Retrograde transvenous thoracic duct embolization for lymphatic leakage after retroperitoneal tumor and lymph node resection: a case report and literature review

BackgroundPostoperative lymphatic leakage is a complication of ineffective conservative treatment for retroperitoneal mass. Herein, we report a case of lymphatic leakage that arose after retroperitoneal tumor resection and that was treated with retrograde transvenous thoracic duct embolization.Case presentationA 28-year-old man with persistent abdominal pain was diagnosed with a large retroperitoneal metastatic tumor measuring 10 cm and a subdiaphragmatic lymph node originating from a testicular tumor. After high orchidectomy and neoadjuvant chemotherapy, the subdiaphragmatic lymph node and retroperitoneal tumor were resected together with the abdominal aorta; the latter was reconstructed using a prosthetic graft. Postoperatively, the patient developed chylothorax. No improvement was observed after conservative treatment that included fasting and somatostatin therapy. The leakage site could not be identified using antegrade lymphangiography of the bilateral inguinal lymph nodes, but was detected using retrograde transvenous lymphangiography. The leakage site was successfully embolized.ConclusionThis case report describes successful treatment with retrograde transvenous thoracic duct embolization for chylothorax following resection of a retroperitoneal tumor and lymph node. This approach is a less invasive and more effective mode of treatment for chylothorax and should be considered before surgical thoracic duct ligation when the leakage point cannot be identified using the antegrade approach.

Adrenal Myelolipoma: Case Report

Introduction: Myelolipomas were first described in 1905 as an adrenal tumor composed of mature fat mixed with myeloid and erythroid cells. Adrenal myelolipomas are the second most common benign type of tumor in the adrenals, following after adrenocortical adenomas. It is mostly discovered incidentally on imaging of abdomen done for non-adrenal related reasons or at autopsy. Symptomatic tumors, growing tumors or tumors larger than 10 cm should be excised surgically. We report the case of 1 male patient presenting with flank pain and upper pole renal masse, treated surgically with adrenalectomy. Myelolipoma was confirmed on histology. Case presentation: a 46-years-old male, with a history of blood hypertension, presented with persistent abdominal discomfort and occasional pain in the right flank region. Physical examination revealed a non-tender palpable mass in the right upper quadrant of the abdomen. The abdominal CT scan revealed a large well-defined retroperitoneal tumor, which compressed and displaced the right kidney downward, the mass was heterogeneous with areas of soft-tissue attenuation. The patient underwent open left adrenalectomy. The pathological study confirmed the diagnosis of adrenal myelolipoma. The differential diagnosis of fat-containing retroperitoneal masses would include retroperitoneal lipoma or liposarcoma and renal angiomyolipoma. Discussion: Adrenal myelolipomas are the second most common adrenal incidentalomas comprising 6 to 16% of adrenal incidentalomas. The size of adrenal myelolipomas is variable and can range from a few millimeters to greater than 10 cm. These are called giant adrenal myelolipomas. The largest adrenal myelolipoma reported to date weighed 6 kg. Extra-adrenal sites for myelolipomas include the retroperitoneum, thorax, and pelvis. Usually unilateral however they can also involve both adrenals. The most common symptoms observed are abdominal discomfort/pain, hypochondrial pain, and flank pain. Ultrasonography (USG), ......

Surgical approach for complete resection of giant retroperitoneal liposarcoma with diaphragmatic hernia via ninth rib thoracotomy.

Resection of a giant retroperitoneal liposarcoma is difficult and technically demanding, especially for large retroperitoneal tumors accompanied by a diaphragmatic hernia. Technically, the open abdominal approach can be time-consuming and difficult to perform, with possible intraoperative complications and other factors bringing psychological and physical difficulties to the patient. This study reports a safe and feasible approach for the complete resection of a large retroperitoneal tumor complicated by a diaphragmatic hernia. A 58-year-old male patient with persistent upper abdominal pain and distension was treated at a local hospital on 4 July 2022. Computed tomography showed a mixed-density mass on the right retroperitoneum, and liposarcoma was considered. On 6 July 2022, the patient was transferred to our hospital for further treatment. Computed tomography showed a mass with low-density fatty shadow in the right adrenal region. The boundary with the right adrenal gland was unclear. The mass was 102mm × 74mm, and the right lobe of the liver was compressed. Insufficiency of the right middle lobe of the liver was seen due to a right diaphragmatic hernia and left mediastinal deviation. We considered the traditional approach for tumor resection via laparotomy, but we opted to perform a comprehensive evaluation first. The tumor was close to the back of the right kidney and liver, causing the diaphragm to rise because of its proximity to these organs. Exposing the tumor through laparotomy would be difficult, making it challenging to remove. The patient had a diaphragmatic hernia and moderate pulmonary dysfunction; therefore, we decided to enter the abdomen through a thoracotomy of the ninth rib. Using our technique, the tumor was easily visualized and completely removed in approximately 30min. The intraoperative blood loss was 100ml, and no postoperative bleeding, pneumothorax, intestinal fistula, infection, or other complications occurred. The transthoracic approach may be a safer and more feasible resection method than the traditional open approach for patients with giant retroperitoneal liposarcoma with a diaphragmatic hernia.

114: Preoperatory and Postoperatory Nutritional Repletion aiming at Intestinal Rehabilitation: A 5-year Prospective Protocol for Large Retroperitoneal Malignant Tumors

114: Preoperatory and Postoperatory Nutritional Repletion aiming at Intestinal Rehabilitation: A 5-year Prospective Protocol for Large Retroperitoneal Malignant Tumors

Large retroperitoneal extraskeletal Ewing’s sarcoma with renal pedicle invasion: a case report

BackgroundExtraskeletal Ewing’s sarcoma (EES) is a rare malignant tumor primarily found in children and young adults. Localized disease can present with nonspecific symptoms such as local mass, regional pain, and increased skin temperature. More severe cases may present with systemic symptoms such as malaise, weakness, fever, anemia, and weight loss. Among these lesions, retroperitoneal sarcomas are relatively uncommon and difficult to diagnose. Since they are usually asymptomatic until large enough to compress or invade the surrounding tissues, most are already advanced at first detection. Traditionally, the treatment of choice is complete surgical resection, sometimes combined with postoperative radiotherapy and chemotherapy. We report a case of EES with left renal artery invasion in the left retroperitoneal cavity successfully treated with transarterial embolization and surgery.Case presentationA 57-year-old woman with a negative family history of cancer presented at our Urology Department with a large left retroperitoneal tumor found by magnetic resonance imaging during the health exam. Physical examination showed a soft abdomen and no palpable mass or tenderness. Imaging studies showed that the tumor covered the entire left renal pedicle, but the left kidney, left adrenal gland, and pancreas appeared tumor free. Since the tumor tightly covered the entire renal pedicle, tumor excision with radical nephrectomy was advised. The patient underwent transarterial embolization of the left renal artery with 10 mg of Gelfoam pieces daily before surgical excision. Tumor excision and left radical nephrectomy were uneventful the day after embolization. Post-operatively, the patient recovered well and was discharged on day 10. The final histopathological analysis showed a round blue cell tumor consistent with an Ewing sarcoma, and the surgical margins were tumor free.ConclusionsRetroperitoneal malignancies are rare but usually severe conditions. Our case report showed that retroperitoneal EES with renal artery invasion could be treated safely with transarterial embolization and surgery.

Primary leiomyosarcoma of the inferior vena cava in a pediatric case: a case report and literature review

BackgroundLeiomyosarcoma is classified as a soft tissue sarcoma. In adults, leiomyosarcoma is the most common malignancy affecting the vascular system; however, vascular leiomyosarcoma in children is extremely rare as most pediatric soft tissue tumors are rhabdomyosarcomas. The survival rate is very low, and incomplete resection is a poor prognostic factor. There is also a high rate of distant recurrence, with the lungs and liver being the most common sites of metastasis. There is no established effective chemotherapy, and complete surgical resection is the only potentially curative treatment for leiomyosarcoma.Case presentationA 15-year-old female patient with no significant medical history presented with severe upper abdominal pain and was admitted. Abdominal contrast-enhanced computed tomography and magnetic resonance imaging showed a large retroperitoneal tumor protruding into the lumen of the inferior vena cava behind the liver and multiple small nodules, and metastasis to the liver was suspected. The tumor was 6 × 4 × 5 cm in diameter, located just behind the hepatic hilar structures, and was suspected to infiltrate into the right portal vein. The tumor was diagnosed as a leiomyosarcoma through an open tumor biopsy. As the multiple liver metastases were located only in the right lobe of the liver on imaging, we performed tumor resection with right hepatectomy and replacement of the inferior vena cava (IVC). The postoperative course was uneventful; however, on postoperative day 51, distant metastatic recurrences were found in the remaining liver and right lung. The patient was immediately started on chemotherapy and trabectedin proved to be the most effective drug in the treatment regimen; however, severe side effects, such as hepatotoxicity, prevented timely administration, and the patient passed away 19 months after surgery.ConclusionsIVC resection and reconstruction combined with right hepatectomy were able to be safely performed even in a pediatric case. To improve the prognosis of leiomyosarcoma with multiple metastases, an effective treatment strategy combining surgical treatment and chemotherapy, including molecularly targeted drugs, should be established as early as possible.

The Use of Extracorporeal Circulation to Facilitate the Resection of a Large Retroperitoneal Sarcoma With Major Vessel Involvement: A Single-Center Review of 20 Cases

To describe the introduction of extracorporeal corporeal oxygenation (ECMO) to facilitate the surgical resection of large retroperitoneal sarcomas involving major vessels and to report preliminary outcomes. A case series. At a tertiary university hospital and state sarcoma center. Patients undergoing retroperitoneal sarcoma resection requiring inferior vena cava (IVC) reconstruction between June 2018 and March 2022. ECMO for retroperitoneal tumor resection requiring IVC reconstruction. A total of 20 patients underwent ECMO-assisted retroperitoneal sarcoma resection and IVC reconstruction. The median age was 60.5 years (IQR 48-69); 15 women and 5 men. The median procedure and ECMO durations were 10.8 hours (IQR 8.5-12.4 hours) and 2.2 hours (IQR 62-218 minutes), respectively. The median intensive care unit and hospital lengths of stay were 4 days (IQR 3-5 days) and 21 days (IQR 14-31 days), respectively. All 20 patients received packed cell transfusions (median 8 per patient [IQR 4-14]); 11 patients required fresh frozen plasma, 6 required platelets, and 11 required fibrinogen supplementation. One patient required recombinant activated factor VII. Sixteen patients experienced acute kidney injury, with 12 patients progressing to chronic kidney disease. Three patients were required to return to the operating room within 7 days, with no returns within the first 24 hours. There was no in-hospital or 30-day mortality. Survival at 3 years was 84%. ECMO for resecting large retroperitoneal tumor resection was introduced successfully and facilitated satisfactory outcomes for many patients who might otherwise have been considered too high risk.

Large retroperitoneal tumor operated for suspected renal cell carcinoma turned out to be primary renal synovial sarcoma: A rare entity

Approximately 5%–10% of soft-tissue tumor comprised synovial sarcoma (SS) and are commonly found in close proximity of large joints affecting young adults. However, SS of the kidney is extremely unusual (1%) and has a poor prognosis. There are three subtypes of primary renal SS (PRSS). The monophasic variant is the most common and shares the same clinical, molecular, and ultrastructural features with other subtypes. Histomorphology with immunohistochemistry (IHC) differentiates it from other sarcomas of kidney and sarcomatoid renal cell carcinoma. Genetic analysis reveals specific SYT-SSX2 (X, 18) translocation. Herein, a case of PRSS (monophasic variant) is presented in a 31-year-old female with right-sided loin pain and huge mass of size (11.9 cm × 9.3 cm × 10 cm) without associated hematuria. It was clinically thought to be renal cell carcinoma. Radical nephrectomy was performed, followed by histopathology and IHC which suggested PRSS. Then, combined chemotherapy of doxorubicin with cyclophosphamide was rendered, and on follow-up, she is doing well with an uneventful course.

Combined surgical treatment of a pinched, unrecoverable, ventral hernia of the anterior abdominal wall and retroperitoneal tumor: a case from the practice of a district surgeon

The study presents the results of the combined surgical treatment of a pinched, unrecoverable ventral hernia of the anterior abdominal cavity, resection of a non-viable pinched loop of the small intestine, and side-to-side anastomosis and removal of a large retroperitoneal tumor. The latter required an incision of the anterior abdominal wall along the white line of the abdomen, from the xiphoid process of the sternum, to the upper border of the pubis, with subsequent plastic surgery of the hernial gate with local tissues.

Reconstruction of caval veins

To study the possibilities and results of reconstruction of caval veins. We analyzed the results of reconstruction of caval veins in 31 patients (19 men and 12 women) including superior vena cava (SVC) in 5 cases and inferior vena cava (IVC) in 26 cases. Penetrating wounds with vascular damage were found in 8 patients. Iatrogenic damage to IVC was observed in 19 patients (nephrectomy for kidney cancer - 2, nephrectomy for secondary kidney wrinkling - 1, echinococcectomy from retroperitoneal space - 1, adrenalectomy for adrenal tumors - 5, right-sided lumbar sympathectomy - 1, resection of abdominal aortic aneurysm - 1, resection of a large retroperitoneal tumor - 6). Iatrogenic damage to SVC occurred in 2 patients during resection of mediastinal tumor. In other 4 cases, elective surgery for mediastinal tumor (1), pancreatic head cancer (2) and liver alveococcosis (1) was accompanied by resection and replacement of caval veins. All interventions for caval vein injury were performed under adequate infusion therapy. Seven (22.6%) patients died. One patient with blunt chest trauma and damage to SVC died during thoracotomy. In another patient, infrarenal IVC was intersected during mobilization of retroperitoneal hydatid cyst that required ligation for vital indications. High venous hypertension below the ligature led to eruption of sutures on the venous stump. The patient died from hypovolemia after additional IVC ligation. Other 5 patients died in early postoperative period without leaving the state of shock. These patients had damage to retrohepatic segment of IVC (1), vascular-organ (1) and iatrogenic (3) injuries. One patient died from pulmonary embolism, two patients - from venous bleeding between the 2nd and the 5th postoperative days. Patients died before reoperations. Two patients with postoperative bleeding underwent redo surgery with favorable outcomes. One patient underwent redo surgery for peritonitis with a favorable result. Thus, 7 (22.6%) patients with caval vein injury died in intraoperative and early postoperative period. Non-specific complications occurred in 4 (12.9%) patients. These events were corrected by conservative measures. Other 24 (77.4%) patients with traumatic and iatrogenic injuries of caval veins were discharged. Caval vein injury is less common event compared to other vascular damages. Nevertheless, this complication is accompanied by severe blood loss, shock and hypovolemia. We can only assume damage to a great vessel in patients with penetrating wounds before surgery and appropriate symptoms of internal bleeding. However, final diagnosis is made during surgery. Hemostasis is a responsible and difficult surgical stage in these patients. There is usually no alternative to reconstructive surgery in these cases. However, ligation is permissible in extremely ill patients and only in infrarenal segment of IVC. Vascular suture is a more acceptable and effective option for reconstruction. However, patch repair is advisable for large defects. In our opinion, this approach is better regarding long-term patency compared to total replacement with synthetic prostheses.

Not just weight loss

Renal cancer (RC) – associated increased calcium is rather frequent among cases of kidney neoplasia; it may be the first sign of presentation that allows the detection of the malignancy; typically representing a poor prognostic factor. Weight loss is part of the panel underling hypercalcemia - related signs, but also of the malignancy – associated phenotype. We aim to introduce a male case who was admitted for a clinical picture (including weight loss) correlated with alarming high calcium levels; the biochemistry anomaly was actually a paraneoplastic syndrome due to RC. When admitted as emergency, the assays revealed hypercalcemia of 16.9 mg/dl (normal levels between 8.4 and 10.2 mg/dl) with low PTH (Parathormone) and high CrossLaps as bone turnover marker of resorption. Intravenous contrast computed tomography showed a large left retroperitoneal tumor (involving the kidney and adrenal gland) with iodophil pattern, and post-contrast heterogeneous structure of 8.06/11.09 cm (axial), 10.58/12.16 cm (coronal-reconstruction), 9.12/10.59 cm (sagittal - reconstruction), also associating a mixt structure and micro-calcifications as well as hypodense areas. Whole body bone scintigram did not show metastasis. The calcium levels were controlled under subcutaneous denosumab 60 mg in addition to IV fluids replacement; then the patient was referred to nephrectomy; postoperative confirmation confirmed a clear cell renal cell carcinoma (T4N1Mx). A normalization of calcium and PTH levels was found immediately after surgery. Unintentional weight loss might become a valuable tool in order to assess a routine biochemistry panel that will detect hypercalcemia. Low PTH represents the next step in order to further look for a tumor. Symptomatic control of hypercalcemia in addition to targeted approach of originating tumor are essential to improve the outcome.

A large retroperitoneal lipoblastoma as an incidental finding: a case report

BackgroundLipoblastoma is a rare benign mesenchymal neoplasm of infancy that most commonly occurs on the extremities and trunk but can arise at variable sites of the body. Retroperitoneal lipoblastomas are particularly rare but can grow to enormous size, and preoperative diagnosis is difficult with diverse, mostly malignant differential diagnoses that would lead to aggressive therapy. Since lipoblastoma is a benign tumor that has an excellent prognosis after resection, correct diagnosis is crucial.Case presentationA case of a large retroperitoneal tumor of a 24-month old infant that was clinically suspicious of a malignant tumor is presented. Due to proximity to the right kidney, clinically most probably a nephroblastoma or clear cell sarcoma of the kidney was suspected. Radiological findings were ambiguous. Therefore, the mass was biopsied, and histology revealed an adipocytic lesion. Although mostly composed of mature adipocytes, in view of the age of the patient, the differential diagnosis of a (maturing) lipoblastoma was raised, which was supported by molecular analysis demonstrating a HAS2-PLAG1 fusion. The tumor was completely resected, and further histopathological workup led to the final diagnosis of a 13 cm large retroperitoneal maturing lipoblastoma. The child recovered promptly from surgery and showed no evidence of recurrence so far.ConclusionAlthough rare, lipoblastoma should be included in the differential diagnoses of retroperitoneal tumors in infants and children, and molecular diagnostic approaches could be a helpful diagnostic adjunct in challenging cases.

Giant abdominopelvic retroperitoneal leiomyoma

Primary giant retroperitoneal leiomyoma are rare pelvic benignomas with about a hundred described in the literature. We present a case of an extremely large abdominopelvic retroperitoneal solid tumor (leiomyoma) in a 43-year nuligravidas, 38 × 30 × 21 cm, weight 8600 g, resolved by tumorectomy with full recovery and fertility preserva-tion.

A case of retroperitoneal dedifferentiated liposarcoma successfully treated by neoadjuvant chemotherapy and subsequent surgery

BackgroundRetroperitoneal liposarcoma (RPLS) is the most commonly observed soft tissue sarcoma in the retroperitoneal space. Although the beneficial effect of chemotherapy for RPLS is controversial, there are some reports that have shown a considerable tumor-suppressive effect of chemotherapy in RPLS. We demonstrate a case of dedifferentiated RPLS, which was initially considered inoperable but was successfully treated by neoadjuvant chemotherapy and subsequent curative resection.Case presentationA 59-year-old female was referred to our hospital with a chief complaint of right lower quadrant abdominal pain. Abdominal computed tomography revealed a large retroperitoneal tumor with a maximum diameter of 11 cm. The tumor involved retroperitoneal major vasculatures, such as the right common iliac vein and artery, as well as the right psoas muscle and femoral nerve. The right ureter was also involved and obstructed by the tumor. A biopsy was performed through the retroperitoneal route, and the tumor was diagnosed as a dedifferentiated liposarcoma with the Fédération Nationale des Centres de Lutte Contre le Cancer grade 3. Because the tumor was highly invasive and complete resection was not feasible, we decided to administer neoadjuvant chemotherapy with doxorubicin and ifosfamide (AI). After completing 6 courses of AI, the tumor size was considerably reduced, and we decided to perform surgery with curative intent. Before laparotomy, femoro-femoral arterial bypass was performed to prepare for the right common iliac artery resection. Thereafter, the patient underwent laparotomy and tumor resection combined with right nephrectomy, resection of the right common iliac artery and vein, and resection of the right psoas muscle and femoral nerve. The postoperative course was uneventful, although the patient needed a walking brace to support her gait. The pathological findings indicated a 99% disappearance of tumor cells. The patient was healthy without any complaints after 1 year of surgery, and a follow-up CT scan revealed no tumor recurrence.ConclusionsTo the best of our knowledge, this is the first report that showed a nearly complete pathological response to AI in dedifferentiated RPLS, which was subsequently completely resected.

En bloc removal of a large leiomyosarcoma arising from the inferior vena cava using retroperitoneal laparoscopic dissection of posterior vessels followed by cavectomy in a thoracoabdominal approach

A 55-year-old Japanese woman had a large retroperitoneal tumor involving the inferior vena cava (IVC) in the right infrahepatic space. We performed en bloc tumor resection with the right kidney and ipsilateral adrenal gland. Because of the large tumor size, we used a retroperitoneal laparoscopic approach for the posterior dissection and performed renal artery ligation. Following open conversion by a thoracoabdominal anterior approach, the tumor was completely removed without major reconstruction. Pathological examination confirmed a leiomyosarcoma arising from the IVC. Retroperitoneal laparoscopic dissection of the posterior side enabled easy and safe surgery even for a large tumor involving the IVC.

88. A Large Primary Retroperitoneal Mucinous Tumor in Pregnancy: A Case Report

Primary retroperitoneal mucinous tumors are rare with only about 50 cases reported in the literature. Of these only 13 cases were primary retroperitoneal mucinous cystic tumors of borderline malignancy. There is no unanimous consensus on the origin of these tumors and due to its extreme rarity, its optimal management and prognosis are uncertain.

PRIMITIVE RETROPERITONEAL TUMORS – GIANT LIPOSARCOMA

Primitive retroperitoneal tumors are formations with varying degrees of severity. They may occur locally, being impressive due to their generous diameters, but they can also invade the entire body. The latest genetic studies have somewhat changed the vision about the etiology of soft tissue tumors, while the immunohistochemical studies offer customized results on recurrence, metastasis and survival rate. At the same time, the importance of the imaging findings is crucial, especially when the localization poses problems regarding the surgical technique and a means of adjuvant therapy must be chosen accordingly. The peculiarity of this case is that we managed to resect the impressively large retroperitoneal malignant tumor with oncological safety margins. We present the case of a patient admitted in 2012 in the Department of General Surgery II of the "Carol Davila" Central Military Emergency Hospital, because of the development of a bulky formation in the right hemiabdomen and subocclusive symptoms. A thorough investigation was performed. The initial suspicion was of a primitive retroperitoneal tumor, the CT aspect indicating a liposarcoma. The total tumoral excision was decided and performed, with the previous checking of the specific areas of metastases. The diagnosis certitude was obtained after sending the 5.5 kg piece to the anatomical pathology examination. Post-operatively, the patient was referred to the oncological service for appropriate adjuvant treatment. So far, the surgical treatment combined with the oncological one has had favorable results.

Large retroperitoneal soft tissue tumor: A cytopathological diagnosis.

Large retroperitoneal soft tissue tumor: A cytopathological diagnosis.

A large retroperitoneal lymphatic malformation successfully treated with traditional Japanese Kampo medicine in combination with surgery

BackgroundCurrent treatment options for lymphatic malformations (LMs) are multimodal. Recently, the effectiveness of treating LMs with Eppikajyutsuto (TJ-28) has been reported. TJ-28 is a kind of oral herbal medicine classified as the traditional Japanese Kampo medicine.Case presentationA 12-year-old girl was admitted to our hospital for intermittent upper abdominal pain. Radiological examinations revealed a large (9.5 × 5.8 × 10.0 cm) retroperitoneal LM, which was suspected to adhering and stretching both pancreas head and duodenum. The large retroperitoneal tumor resection might induce involving complications because of the size and the location. Therefore, we used TJ-28 in order to diminish the tumor size before surgery. The patient received oral doses of 7.5 g/day (2.5 g × 3 times/day) of TJ-28. Six months after the medication, the tumor decreased markedly to 3.5 × 1.5 × 1.2 cm in size. Thereafter, the mass was sub-totally resected (95%) via a 3 cm trans-umbilical incision without any surgical complications.ConclusionsWe reported a case of successfully treated retroperitoneal LM with the combination treatment of TJ-28 and surgery. Based on our experience, this TJ-28 treatment option may be very useful in treating cases of LMs having surgical difficulties because of size and/or location.