Staging and Restaging Pediatric Abdominal and Pelvic Tumors: A Practical Guide.

The most common abdominal malignancies diagnosed in the pediatric population include neuroblastoma, Wilms tumor, hepatoblastoma, lymphoma, germ cell tumor, and rhabdomyosarcoma. There are distinctive imaging findings and patterns of spread for each of these tumors that radiologists must know for diagnosis and staging and for monitoring the patient's response to treatment. The multidisciplinary treatment group that includes oncologists, surgeons, and radiation oncologists relies heavily on imaging evaluation to identify the best treatment course and prognostication of imaging findings, such as the image-defined risk factors for neuroblastomas, the PRETreatment EXtent of Disease staging system for hepatoblastoma, and the Ann Arbor staging system for lymphomas. It is imperative for radiologists to be able to correctly indicate the best imaging methods for diagnosis, staging, and restaging of each of these most prevalent tumors to avoid inconclusive or unnecessary examinations. The authors review in a practical manner the most updated key points in diagnosing and staging disease and assessing response to treatment of the most common pediatric abdominal tumors. ©RSNA, 2024 Supplemental material is available for this article.

Response Evaluation Criteria in Gastrointestinal and Abdominal Cancers: Which to Use and How to Measure.

As the management of gastrointestinal malignancy has evolved, tumor response assessment has expanded from size-based assessments to those that include tumor enhancement, in addition to functional data such as those derived from PET and diffusion-weighted imaging. Accurate interpretation of tumor response therefore requires knowledge of imaging modalities used in gastrointestinal malignancy, anticancer therapies, and tumor biology. Targeted therapies such as immunotherapy pose additional considerations due to unique imaging response patterns and drug toxicity; as a consequence, immunotherapy response criteria have been developed. Some gastrointestinal malignancies require assessment with tumor-specific criteria when assessing response, often to guide clinical management (such as watchful waiting in rectal cancer or suitability for surgery in pancreatic cancer). Moreover, anatomic measurements can underestimate therapeutic response when applied to molecular-targeted therapies or locoregional therapies in hypervascular malignancies such as hepatocellular carcinoma. In these cases, responding tumors may exhibit morphologic changes including cystic degeneration, necrosis, and hemorrhage, often without significant reduction in size. Awareness of pitfalls when interpreting gastrointestinal tumor response is required to correctly interpret response assessment imaging and guide appropriate oncologic management. Data-driven image analyses such as radiomics have been investigated in a variety of gastrointestinal tumors, such as identifying those more likely to respond to therapy or recur, with the aim of delivering precision medicine. Multimedia-enhanced radiology reports can facilitate communication of gastrointestinal tumor response by automatically embedding response categories, key data, and representative images. ©RSNA, 2024 Test Your Knowledge questions for this article are available in the supplemental material.

Management of Abdominal Paraganglioma: A Single Center's Experience.

Background and Objectives: Paragangliomas (PGLs) are rare neuroendocrine extra-adrenal tumors that could be secreting mass. The symptoms are the typical triad of paroxysmal headache, hypertension and sweating, but could also be accompanied by symptoms involving multiple organs. Surgery is the gold standard treatment for both PGLs and pheochromocytomas (PHEOs). Material and Methods: We used a computerized endocrine surgery registry to record the demographic and clinical data of 153 patients who underwent surgery for PPGL between 2010 and 2023 at our hospital. Results: Thirteen patients (8.43%) with paragangliomas underwent surgery at our institute. Five patients presented symptomatic syndrome. Preoperative investigations included enhanced abdominal CT (nine patients) and enhanced MRI (seven patients). In cases of suspicious mass, we performed 131I-MIBG scans (two patients) or 68GA-DOTATOC PET-CT scans (11 patients). Laparoscopic approach was used in four cases (30.7%) and abdominal laparotomy in the other nine (69.3%). Biochemical tests were performed on all patients. Conclusions: In this retrospective study, we discuss the multidisciplinary management in our institute of this rare disease, from its challenging diagnosis to the surgical strategy for PGLs. Laparoscopic surgery is the gold standard, but a tailored approach should be adopted for each patient.

Desmoplastic small round cell tumor of the abdomen: A case report.

RATIONALE: Desmoplastic small round cell tumor (DSRCT) is a rare malignant tumor with poor prognosis, usually involving the peritoneum. There are currently no standardized treatment approaches. This study helped to further advance our understanding of DSRCT, and help to guide therapy. PATIENT CONCERNS: The patient, a 19-year-old male, presented with left-sided back pain with no obvious cause and occasional abdominal pain, and underwent abdominal electron computed tomography examination in our hospital suggesting consideration of small bowel mesenchymal tumor with possible multiple implantation metastasis in the abdominopelvic cavity. DIAGNOSES: After surgical treatment, the pathology report suggested a DSRCT, and immunohistochemistry and fluorescence in situ hybridization revealed EWSR1-WT1 gene rearrangement. Lung computer tomography and abdominal magnetic resonance imaging performed half a month later showed multiple solid nodules on the proximal septal surface of the right lung base, right posterior cardiac/right anterior inferior vena cava nodules, and multiple nodules in the abdominopelvic cavity, omenta, peritoneum, and around the liver or liver, all of which were considered as metastatic foci. INTERVENTIONS AND OUTCOMES: Patient received 5 cycles of chemotherapy after surgery. The review results showed a smaller size than before. Currently, he continues to receive treatment. LESSONS: The reported case has raised awareness of the importance of DSRCT in the treatment of chemotherapy, including its role in the differential diagnosis of abdominal tumors.

Abdominal Tumors: Wilms, Neuroblastoma, Rhabdomyosarcoma, and Hepatoblastoma.

Pediatric cancer patients have improved outcomes over the past several decades leading to a greater number of survivors living well into adulthood. Owing to their increased longevity, adult care providers are encountering childhood cancer survivors with greater frequency in their clinics and hospitals. Childhood cancer treatments are associated with varied and significant systemic complications that either persist or develop well into adulthood, including secondary malignancies, cardiomyopathies, and adhesive disease that can complicate even the simplest operation. This article reviews four of the most common solid abdominal tumors in the pediatric population and the long-term sequelae of their respective treatment regimens.

Cytokeratin-positive Malignant Tumor in the Abdomen With EWSR1/FUS-CREB Fusion: A Clinicopathologic Study of 8 Cases.

ATF1, CREB1, and CREM, which encode the CREB family of transcription factors, are fused with EWSR1 or FUS in human neoplasms, such as angiomatoid fibrous histiocytoma. EWSR1/FUS-CREB fusions have recently been reported in a group of malignant epithelioid tumors with a predilection to the peritoneal cavity and frequent cytokeratin expression. Here, we studied 8 cytokeratin-positive abdominal malignancies with these fusions for further characterization. The tumors affected males (15 to 76 y old) and presented as intra-abdominal masses with concurrent or subsequent peritoneal dissemination, ascites, and/or metastases to the liver or lymph nodes. Four patients died of the disease within 18 to 140 months. Cases 1 to 5 showed multinodular growth of monomorphic epithelioid cells with focal serous cysts. Lymphoplasmacytic infiltration was prominent and was associated with systemic inflammatory symptoms. Two patients suffered from membranous nephropathy with nephrosis. The tumors displayed partly overlapping phenotypes with malignant mesothelioma, including diffuse strong expression of AE1/AE3 and WT1 and membranous positivity of sialylated HEG1, although calretinin was negative. Case 6 showed similar histology to cases 1 to 5, but expressed smooth muscle actin diffusely, lacked WT1 and HEG1, and harbored prominent pseudoangiomatous spaces. Cases 7 and 8 displayed dense growth of small oval to short spindle cells, with occasional molding and minor swirling, superficially resembling small cell carcinoma. Lymphoplasmacytic infiltration was not observed. The tumors were positive for AE1/AE3 and CD34 (focal), whereas calretinin, WT1, and HEG1 were negative. The detected fusions were FUS-CREM (n=4), EWSR1-ATF1 (n=2), EWSR1-CREB1 (n=1), and EWSR1-CREM (n=1). We confirmed the prior observation that these tumors do not fit perfectly with known entities and provided additional novel clinicopathologic information. The tumors require wider recognition because of more aggressive behavior than angiomatoid fibrous histiocytoma despite similar genetics, and potential misdiagnosis as unrelated diseases, such as neuroendocrine neoplasms.

Hemostatic Management in an Infant With Neuroblastoma and Severe Hemophilia B With Extended Half-life Recombinant Factor IX Fusion Protein.

In the rare co-occurrence of childhood cancer and severe hemophilia, hemostatic management is of paramount therapeutic importance. We present the case of an 11-month-old boy with severe congenital hemophilia B, who was diagnosed with metastatic high-risk neuroblastoma. He consequently developed paraneoplastic coagulopathy with life-threatening tumor hemorrhage and intracranial hemorrhage, showing central nervous system relapse. Management consisted of factor IX replacement with extended half-life factor IX fusion protein, adjusted to bleeding risk. Additional interventions included factor XIII, fibrinogen, fresh frozen plasma, tranexamic acid, and platelet transfusions. The half-life of factor IX products was markedly reduced requiring close factor IX monitoring and adequate replacement. This intensified treatment allowed chemotherapy, autologous stem cell transplantation, and GD2 antibody immune therapy without bleeding or thrombosis.

A single-centre ten-year retrospective cohort study of malignant small bowel obstruction.

INTRODUCTION: Management of malignant small bowel obstruction (mSBO) is challenging. The decision to perform an operation evaluates the perceived chance of success against a patient's fitness for operation. The aim of this study was to characterise the mSBO patient population in a tertiary UK centre and assess the patient's treatment pathway including use and effects of palliative surgery, total parenteral nutrition (TPN), Gastrografin and dexamethasone as well as preoperative stratification. METHODS: Patients were included if they had mSBO confirmed on computed tomography imaging due to a primary or metastatic neoplasm. Data were collected on pathway and management, and Cox proportional hazard methods were utilised to observe effects on survival. RESULTS: Ninety-four patients were included, with 104 inpatient episodes. Mean age was 67.4 (SD 13.7), with 57 (60.6%) females. Most (89.4%) had only one admission for mSBO. Eighty-four (89.4%) patients died over the ten-year period, 18 (17.3%) within 30 days of admission. Fifty patients (53.1%) underwent operative management: 70% bypass, 24% stoma formation and 6% open-close laparotomies. Log rank testing of survival probability analysis was significant (p = 0.00018), with 50% survival probability at 107.32 days for operative management and 47.87 days for non-operative. DISCUSSION AND CONCLUSION: Operative management forms part of the treatment pathway for a significant proportion of patients with mSBO, offering a survival benefit, though quality of survival is not known. Case selection is good, with few open-close laparotomies. Trials of non-operative interventions such as Gastrografin and dexamethasone are not utilised fully.

Imaging for Staging of Pediatric Abdominal Tumors: An Update, From the AJR Special Series on Cancer Staging.

The three most common pediatric solid tumors of the abdomen are neuroblastoma, Wilms tumor, and hepatoblastoma. These embryonal tumors most commonly present in the first decade of life. Each tumor has unique imaging findings, including locoregional presentation and patterns of distant spread. Neuroblastoma, Wilms tumor, and hepatoblastoma have unique staging systems that rely heavily on imaging and influence surgical and oncologic management. The staging systems include image-defined risk factors for neuroblastoma, the Children's Oncology Group staging system for Wilms tumor, and the pretreatment extent of tumor system (PRETEXT) for hepatoblastoma. It is important for radiologists to be aware of these staging systems to optimize image acquisition and interpretation. This article provides a practical and clinically oriented approach to the role of imaging in the staging of these common embryonal tumors of childhood. The selection among imaging modalities, key findings for determining tumor stage, and the role of imaging in posttreatment response evaluation and surveillance are discussed. Recent updates to the relevant staging systems are highlighted with attention to imaging findings of particular prognostic importance. The information presented will help radiologists tailor the imaging approach to the individual patient and guide optimal oncologic management.

Port-Site Metastasis in Gynecological Malignancies.

BACKGROUND: Minimally invasive oncologic surgery has become the standard of care in many gynecologic cancers. While laparoscopic surgery provides many benefits to patients, such as faster recovery, there are unique challenges associated with minimally invasive techniques. Port-site metastasis is a rare complication after laparoscopic oncologic surgery in management of gynecologic malignancies. METHODS: We present the case of a 44-year-old female with isolated port-site recurrence following laparoscopic radical hysterectomy with node-negative, clinical stage IB1 cervical adenocarcinoma. In addition, we provide an updated review of the literature on management and oncologic outcomes of port-site metastasis. CONCLUSION: Port-site metastasis prevention necessitates a better understanding of underlying risk factors and pathophysiology in order to optimize outcomes. Future studies are needed on risk-reducing strategies and standardization of management for port-site metastasis.

Abdominal Recurrence after Robotic Nephron-Sparing Surgery for Wilms Tumor in an Adult Patient.

A 31-year-old man was referred to an adult urologist for a renal polar mass that measured 7.2 cm in maximum diameter. Robotic assisted complete tumor excision for suspicious renal cell carcinoma was carried out, preserving the rest of the left kidney. Histopathology showed a Wilms tumor (WT) with positive margins. No postoperative therapy was made, and the patient shortly presented an abdominal recurrence. The patient was referred to our pediatric oncology unit; he received preoperative chemotherapy, followed by surgery (completion nephrectomy and removal of neoplastic deposits in the omentum and parietal peritoneum), postoperative chemotherapy, and abdomen radiotherapy. He is well at the 5-year follow-up. Peritoneal dissemination after laparoscopic nephron-sparing surgery (NSS) in a child with a 10-cm WT was previously reported. We suggest open NSS for large WT may be safer than laparoscopic or robotic NSS because carbon dioxide pneumoperitoneum and traumatic handling of tumor may predispose to tumor cell migration. An abdominal WT relapse in adults can be salvaged by multimodal therapy recommended by current pediatric WT guidelines.

An assessment of the use of patient reported outcome measurements (PROMs) in cancers of the pelvic abdominal cavity: identifying oncologic benefit and an evidence-practice gap in routine clinical practice.

BACKGROUND: Patient reported outcome measurements (PROMs) are emerging as an important component of patient management in the cancer setting, providing broad perspectives on patients' quality of life and experience. The use of PROMs is, however, generally limited to the context of randomised control trials, as healthcare services are challenged to sustain high quality of care whilst facing increasing demand and financial shortfalls. We performed a systematic review of the literature to identify any oncological benefit of using PROMs and investigate the wider impact on patient experience, in cancers of the pelvic abdominal cavity specifically. METHODS: A systematic review of the literature was conducted using MEDLINE (Pubmed) and Ovid Gateway (Embase and Ovid) until April 2020. Studies investigating the oncological outcomes of PROMs were deemed suitable for inclusion. RESULTS: A total of 21 studies were included from 2167 screened articles. Various domains of quality of life (QoL) were identified as potential prognosticators for oncologic outcomes in cancers of the pelvic abdominal cavity, independent of other clinicopathological features of disease: 3 studies identified global QoL as a prognostic factor, 6 studies identified physical and role functioning, and 2 studies highlighted fatigue. In addition to improved outcomes, a number of included studies also reported that the use of PROMs enhanced both patient-clinician communication and patient satisfaction with care in the clinical setting. CONCLUSIONS: This review highlights the necessity of routine collection of PROMs within the pelvic abdominal cancer setting to improve patient quality of life and outcomes.

Endoscopic Ultrasound-Guided Ethanol Injection Associated with Trans-arterial Embolization of a Giant Intra-abdominal Cavernous Hemangioma: Case Report and New Therapeutic Option.

BACKGROUND: Cavernous hemangiomas are congenital hamartomatous lesions that originate from mesodermal tissue composed of dilated blood vessels. Abdominal pain and palpable mass are the most common presenting symptoms. The different types of treatment for symptomatic patients remain controversial. However, surgical resection is always the most preferred method when possible. To date, there are no reports of endoscopic ultrasound-guided (EUS-guided) absolute ethanol injection as a treatment for such disease when surgery is not an option. CASE PRESENTATION: A 19-year-old girl with giant cavernous intra-abdominal hemangioma extending to the hepatic hilum, also affecting the gastric wall and occupying the entire supra-mesocolic cavity, initially presents with upper gastrointestinal bleeding and loss of 20 kg in 1 year (BMI = 18 kg/m2). Percutaneous angiography identified a mass with arterial blood supply by the left gastric artery that was embolized. After re-bleeding, an alternative treatment with EUS-guided injection of alcohol was proposed once resection was not feasible without major risks to a young patient. This procedure was repeated 15 and 45 days after the initial treatment, with the ethanol injection of 25 cc and 15 cc, respectively. On the second and third procedure dates, there was evident regression of the hemangioma. On the third procedure, it was possible to identify all anatomic structures that were not clear on the first EUS. After 45 days of last injection, abdominal CT and EUS showed notorious regression of the lesion. Eight months later, abdominal CT showed only a remnant lesion in the hepatogastric ligament with 129 cm3 on volumetry (87% lower in comparison to the initial image), and the patient remains asymptomatic with BMI of 26. In the most recent follow-up CT, 4 years and 2 months after first treatment, the patient presents with a slight increase in the hemangioma-now with 183 cm3 on volumetry. CONCLUSION: Transgastric EUS-guided ethanol injection in the treatment of giant intra-abdominal cavernous hemangioma can provide good outcomes without major complications and can be repeated if necessary.

Metastatic Pheochromocytomas and Abdominal Paragangliomas.

CONTEXT: Pheochromocytomas and paragangliomas (PPGLs) are believed to harbor malignant potential; about 10% to 15% of pheochromocytomas and up to 50% of abdominal paragangliomas will exhibit metastatic behavior. EVIDENCE ACQUISITION: Extensive searches in the PubMed database with various combinations of the key words pheochromocytoma, paraganglioma, metastatic, malignant, diagnosis, pathology, genetic, and treatment were the basis for the present review. DATA SYNTHESIS: To pinpoint metastatic potential in PPGLs is difficult, but nevertheless crucial for the individual patient to receive tailor-made follow-up and adjuvant treatment following primary surgery. A combination of histological workup and molecular predictive markers can possibly aid the clinicians in this aspect. Most patients with PPGLs have localized disease and may be cured by surgery. Plasma metanephrines are the main biochemical tests. Genetic testing is important, both for counseling and prognostic estimation. Apart from computed tomography and magnetic resonance imaging, molecular imaging using 68Ga-DOTATOC/DOTATATE should be performed. 123I-MIBG scintigraphy may be performed to determine whether 131I-MIBG therapy is a possible option. As first-line treatment in patients with metastatic disease, 177Lu-DOTATATE or 131I-MIBG is recommended, depending on which shows best expression. In patients with very low proliferative activity, watch-and-wait or primary treatment with long-acting somatostatin analogues may be considered. As second-line treatment, or first-line in patients with high proliferative rate, chemotherapy with temozolomide or cyclophosphamide + vincristine + dacarbazine is the therapy of choice. Other therapies, including sunitinib, cabozantinib, everolimus, and PD-1/PDL-1 inhibitors, have shown modest effect. CONCLUSIONS: Metastatic PPGLs need individualized management and should always be discussed in specialized and interdisciplinary tumor boards. Further studies and newer treatment modalities are urgently needed.

Thoracic duct lymphangioma and chylous ascites in a 24-year-old female

Lymphangiomas are rare and correspond to 0.7% to 4.0% of mediastinal tumors, and isolated mediastinal location occurs in 1% of cases. They are benign tumors that originate from a congenital malformation of the lymphatic vessels and are diagnosed more frequently in children less than 2 years of age. Chylous ascites is a clinical manifestation of thoracic duct lymphangioma and is composed of lymph accumulation caused by dilation of this lymphatic channel. It appears milky in the peritoneal cavity, containing triglyceride levels higher than 200 mg/dl. We report the case of a young patient with chylous ascites and lymphangioma of the thoracic duct, who was conservatively treated with octreotide and a low-fat diet with medium-chain triglycerides.

Does a selective surgical approach to malignant bowel obstruction help in palliative care patients?

BACKGROUND: Malignant bowel obstruction (MBO) is a condition secondary to intra-abdominal metastatic spread of advanced-stage tumors. There is no consensus for the treatment approach of MBO. This study aims to present the results of medical treatment and palliative surgery in patients diagnosed with MBO. METHODS: The patients who were treated for advanced-stage tumors between 2010 and 2017 and for whom consultation was requested from the surgical clinic for MBO symptoms were identified. A selective approach together with palliative care for the indication of surgery was instituted. The patients with surgical treatment and medical treatment were compared concerning survival, oral food intake and symptom relief. RESULTS: Seventy-six patients (30 female, 46 male) aged 60.5±12.8 years (range: 27-88) were included in this study. Forty-eight of the patients (64.9%) underwent surgical treatment, while 28 (35.1%) had medical treatment. Although the patients with surgery had longer duration of stay in the hospital (median 16 days vs. 4 days) (p<0.001) and higher complication rates (27.1% vs. 3.5%) compared to medically treated patients; the restoring oral food intake was better (97.9% vs. 78.6%) (p=0.005) and the survival was longer (105 days vs. 43 days). CONCLUSION: This study revealed that surgical treatment resulted in better outcomes for life quality parameters in highly selected patients with malignant bowel obstruction evaluated by multidisciplinary team, including palliative care.

The efficacy and safety of probiotics for prevention of chemoradiotherapy-induced diarrhea in people with abdominal and pelvic cancer: A systematic review and meta-analysis based on 23 randomized studies.

BACKGROUND AND AIMS: We evaluated the efficacy and safety of probiotics for prevention of chemoradiotherapy-induced diarrhea in patients with abdominal or pelvic cancer. METHODS: We searched the Cochrane Library, PubMed, EMBASE, Web of Science, Chinese National Knowledge Infrastructure (CNKI), Wanfang, and VIP databases up to August 2019. We also hand searched the citation lists of included studies and previous systematic reviews identified to identify further relevant trials. The primary outcome was the incidence of chemoradiotherapy-induced diarrhea of all grades. The secondary outcomes were improvement of antidiarrheal medication use, stool form (Bristol scale), response rate, and adverse events (AEs). Diarrhea was graded according to the Common Toxicity Criteria system. Two reviewers assessed trial quality and extracted data independently. The included studies were analyzed using Review Manager ver. 5.2. RESULTS: Twenty-three randomized, placebo-controlled studies (N = 2570 participants) were included in the efficacy assessment. The incidence of all diarrhea (risk ratio [RR] 0.16; 95% confidence interval [CI] 0.51-0.73), grade ≥ 3 diarrhea (RR 0.36; 95% CI 0.18-0.72), and grade ≥ 2 diarrhea (RR 0.65; 95% CI 0.54-0.78), but not that of grade ≤ 2 diarrhea (RR 1.07; 95% CI 0.95-1.21), was significantly reduced in the probiotics compared to the placebo groups. No significant increase in the incidence of AEs was found in the probiotics group, although four studies reported a variety of AEs. CONCLUSIONS: Probiotics prevented chemoradiotherapy-induced diarrhea, particularly high-grade diarrhea. Probiotics rarely cause AEs.

Incidence, treatment and outcome of abdominal metastases in extremity soft tissue sarcoma: Results from a multi-centre study.

BACKGROUND AND OBJECTIVES: Abdominal metastases (AM) from soft tissue sarcoma (STS) are rare and prognosis is poor. The aims of the study were to (a) identify risk factors for the development of AM and to (b) investigate the outcome of AM-patients. METHODS: Seven-hundred-sixty-nine STS-patients with localised disease at diagnosis treated at three tumour centres (2000-2016) were retrospectively included (409 males; mean age, 55.6 years [range, 8-96 years]; median follow-up, 4.1 years [interquartile-range, 2.5-6.6 years]). RESULTS: Two-hundred-two patients (26.3%) developed secondary metastases, and 24 of them AM (3.1%). Ten patients developed first AM (FAM) after a mean of 2.4 years and 14 patients late AM (LAM, after being diagnosed with metastases to other sites) after a mean of 2.0 years. Patients with liposarcoma had a significantly higher risk of developing AM (P = .007), irrespective of grading. There was no difference in post-metastasis-survival (PMS) between patients with AM at any time point and those with metastases to other sites (P = .585). Patients with LAM or FAM showed no difference in post-abdominal-metastasis-survival (P = .884). CONCLUSIONS: Survival in patients with AM is poor, irrespective of whether they develop secondarily to other metastases or not. Patients at high-risk of AM (ie, liposarcoma) may be followed-up regularly by abdominal-ultrasound/CT.