Pediatric Outcomes After Robotic Median Arcuate Ligament Release, Celiac Ganglionectomy, and Lymphadenectomy.

BACKGROUND: Median arcuate ligament syndrome (MALS) is a neurovascular disorder characterized by gastrointestinal symptoms due to celiac artery and ganglion compression. Limited literature primarily focuses on adults. This study aims to characterize clinical and histopathologic findings in pediatric MALS. METHODS: Patients <18 years undergoing robotic MAL release, celiac ganglionectomy, and lymphadenectomy from 2020 to 2024 were evaluated. Clinical and histopathologic outcomes were analyzed. RESULTS: Twelve patients met inclusion criteria and were reviewed (15.7 ± 1.2 years, 75% female, BMI 20.9 [18.6-24.0] kg/m2). Comorbidities included depression/anxiety (83%), postural orthostatic tachycardia syndrome (POTS) (50%), gastroesophageal reflux disease (GERD) (50%), nutrition support (50%), mast cell activation syndrome (MCAS) (42%), hypermobile Ehlers-Danlos syndrome (hEDS) (42%), other vascular compression syndromes (33%). All patients who received preoperative celiac plexus block had temporary symptom relief (10/10). Mean operative time was 119.7 ± 22.4 min. No intraoperative complications, 30-day readmissions, reoperations, or complications occurred. Histopathologically, 92% had fibroadipose tissue, 100% had reactive lymph nodes, none had intraparenchymal nerves, and one had lipogranulomas. Median fibrosis scores were 1 [0.5-2] on H&E and 2 [1-2] on trichrome. Fibrosis score severity was not significantly associated with symptom improvement (χ2 = 3.67, p = 0.16). Median postoperative celiac artery velocity was 258.5 [192.5-350.5] cm/s with a median change of -80.5 [-106.1-+82.8] cm/s from preoperative 308.0 [229.3-344.0] cm/s (S = -5.0, p = 0.55). Those with lower preoperative velocities were more likely to have symptom improvement postoperatively (S = 19, p = 0.04). MALS symptoms improved in 83%; however, despite reporting "MALS pain" was improved, 64% (all female) had other comorbidities such as POTS, MCAS, hEDS, and colonic dysmotility contributing to other ongoing symptoms. CONCLUSION: Robotic MALS surgery is safe and effective in pediatrics. Clinical improvement rates and fibrosis scoring are comparable to adults; however, findings reveal challenges with multiple comorbidities contributing to separate symptoms that may continue postoperatively, particularly in females. We recommend a multidisciplinary team approach in addressing comorbidities and optimizing medical and surgical care. LEVEL OF EVIDENCE: IV.

First description of a histopathologic grading system and relationship to outcomes after robotic median arcuate ligament release with celiac ganglionectomy and lymphadenectomy.

BACKGROUND: Two dominating theories regarding median arcuate ligament syndrome include vascular and neurogenic etiologies from celiac artery and ganglion compression, respectively. Celiac ganglionectomy is not routine during surgery, and specimens are rarely excised; therefore, the extent of nerve involvement and histopathology are unknown. Our study aims to characterize histopathologic findings in median arcuate ligament syndrome, establish a histopathologic grading system, and correlate with clinical outcomes. METHODS: Robotic median arcuate ligament release, celiac ganglionectomy, and lymphadenectomy were performed with specimens excised and stained using hematoxylin & eosin, trichrome, and S100. Neurofibrosis, adiposity, and reactive changes were described, a grading scale was developed, and results were analyzed with clinical outcomes. RESULTS: Fifty-four patients were evaluated, of whom 36 met inclusion criteria (81% female, 34.9 [25.9-47.5] years, body mass index 23.5 [19.6-28.1] kg/m2). Histopathologic evaluation revealed fibrosis (hematoxylin & eosin and trichrome median score 1.5 [0-2.5]), reactive lymphadenopathy (89%), intraparenchymal nerves (31%), and lipogranulomas (31%). Greater fibrosis was associated with a lack of preoperative celiac plexus block relief (100% vs. 30%, P = .044) and lower postoperative celiac artery velocities (198 vs 323 cm/s, P = .02). Intraparenchymal nerves were associated with greater decreases in pre to postoperative velocities (161 vs 84 cm/s, P = .037). Symptoms improved in 28 patients (78%). CONCLUSION: We developed the first histopathologic grading system and identified unique findings of intraparenchymal nerves and lipogranulomas. Histopathologic abnormalities were associated with objective improvement and symptomatic relief postoperatively. These findings support nerve compression and inflammation as predominant contributors to median arcuate ligament syndrome pain, celiac ganglia resection to treat symptoms, and continued histopathologic analysis to better elucidate median arcuate ligament syndrome etiology.

Sporadic Renal Hemangioblastoma: A Case Report of a Rare Entity.

Hemangioblastoma, also known as capillary hemangioblastoma, is a rare benign mesenchymal tumor commonly found in the central nervous system (CNS). It can also manifest in various organs, including the kidney. Renal hemangioblastoma (RH) is often associated with Von Hippel-Lindau (VHL) disease, but sporadic occurrences are observed infrequently. While RH is usually asymptomatic, it can also cause abdominal pain and hematuria. In this study, we present a case of an elderly patient without history of VHL but complaining of abdominal pain for three days. Serological evaluations were unremarkable, and a CT scan identified a 2.4 cm mixed solid-cystic mass lesion on the left kidney's superior aspect. The patient subsequently underwent a biopsy followed by lesion ablation. Microscopic analysis revealed sheets of eosinophilic cells with ovoid nuclei, showing focal rhabdoid and spindle cell features, with an intricate capillary network. Focal nuclear atypia without necrosis or mitosis was noted. Immunohistochemistry (IHC) demonstrated positive staining for inhibin, S100, PAX8, and vimentin, along with patchy positivity for CD10 and RCC. Negative staining was observed for cytokeratin AE1/AE3, CK7, EMA, CK8/18, desmin, and HMB-45. The overall morphological characteristics and distinct IHC markers were consistent with RH. Although its pathogenesis remains unclear because of its rarity, distinguishing RH from renal cell carcinoma is crucial. IHC markers facilitate differentiation among lesions. The preferred treatment involves ablation or partial nephrectomy. Further assessment for possible VHL syndrome is essential, considering the distinct management approaches for sporadic and VHL-linked RH.

Sclerosing Angiomatoid Nodular Transformation of the Spleen: A Report of Rare Case and Literature Review.

Sclerosing angiomatoid nodular transformation (SANT) is a benign vascular lesion of the spleen with uncertain etiology. It predominantly affects women between the ages of 30 and 60 years. Clinically, it is asymptomatic or can cause abdominal pain, but usually discovered incidentally on imaging, which can identify a mass but may not provide a definitive diagnosis. In uncertain vascular lesions, there is always a risk of spontaneous rupture of large vessels and the potential for spreading malignancy. Hence, the final diagnosis is rendered on microscopy after splenectomy. A middle-aged female came to the clinic complaining of abdominal pain. Radiology showed a solid splenic mass and the patient underwent splenectomy. Gross examination showed a 3 cm white firm mass with focal hemorrhage. Microscopy revealed multiple nodules of variable sizes surrounded by fibrosclerotic stroma. The nodules showed round to slit-like vascular spaces with numerous red blood cells. The internodular stroma consisted of dense fibrous tissue with scattered plump myofibroblasts and lymphoplasmacytic inflammatory cells. These distinctive features lead to the diagnosis of SANT. SANT possesses characteristic histologic features with distinctive immunohistochemistry (IHC). IHC reveals three different types of vessels within the nodules as follows: (1) small veins (CD34-, CD31+, CD8-), (2) sinusoids (CD34-, CD31+, CD8+), and (3) capillaries (CD34+, CD31+, CD8-). All three types of vessels are negative for CD21/CD35 and CD68. Hemangioma and littoral cell angioma are two frequent vascular tumors in the spleen that should be considered differential diagnoses. Both lesions lack the microscopic features of SANT and have only a single type of vessel. The vessels in hemangioma are (CD31+, CD34+, CD8-), while in littoral cell angioma they are (CD31+, CD34-, CD8-, CD21+, CD68+). There are no specific clinical or radiologic findings for SANT. It is important to recognize these characteristic features and to differentiate them from other benign and malignant lesions, such as angiosarcoma. A thorough histopathologic examination and IHC are helpful in making the correct diagnosis.

Colorectal Adenocarcinoma Derived From Mature Cystic Teratomas: A Case Report With Review of the Literature.

Mature cystic teratomas (MCTs) are the most common benign ovarian germ cell neoplasms in women of reproductive age. Rarely, somatic malignancies arise from MCTs, the most common being squamous cell carcinoma. Adenocarcinomas are less common and colorectal adenocarcinomas are extremely rare. We present a case of somatic adenocarcinoma of colorectal type which may pose challenges in diagnosis and treatment. A middle-aged female presented to the Emergency Department with lower abdominal pain. CT scan revealed an 11 cm sharply demarcated left pelvic mass. Laparoscopy showed a left ovarian mass with torsion, a smooth external surface, and thick brownish contents. An intraoperative evaluation was consistent with an adenocarcinoma. Permanent histopathology revealed adenocarcinoma of colorectal phenotype with necrosis. Additional evaluation of the cyst showed benign colonic epithelial lining. The immunohistochemistry (IHC) profile of positive CDX2 and CK20 and negative PAX8, CK7, ER, and PR suggested a colorectal-type somatic adenocarcinoma arising from the MCT and was staged as IA, after negative endoscopic findings. Due to their rarity and atypical symptoms, distinguishing metastatic tumors from MCT-derived somatic malignancies is a challenging process. CT scan and serum tumor markers can be helpful but are not definite. Thorough clinical evaluation and proper staging are necessary after pathologic evaluation. Extensive sampling and IHC can further characterize the origin of the tumor. Diligent sampling and a high index of suspicion in this case clinched the correct diagnosis and clinical management. The patient is being treated for stage IA ovarian cancer as opposed to stage IV metastatic colorectal cancer.