RESUMO
There is a relative paucity of literature on abdominal compartment syndrome (ACS) in children compared to adults and even less describing ACS in pediatric oncologic patients. We present this case of ACS in a 14-year-old patient to highlight the acuity of lethal consequences despite swift adequate management. Our patient is a 14-year-old male with a history of non-verbal autism and large synovial sarcoma of the left chest wall. He was admitted for scheduled inpatient chemotherapy and radiation. On day 3 of admission, the patient's clinical condition rapidly deteriorated, and a surgical abdomen was found on the exam. In the operating room (OR), massive gaseous distention of the stomach, small intestines, and colon were noted. A loop of small bowel was under such high pressure that the force of evisceration sheared the bowel from the associated mesentery. Due to the severity of the dilated bowel loops, we could not return the eviscerated bowel back inside the abdomen, which led us to leave the Abthera wound vac as sole coverage. The patient was transferred to the PICU, and medical treatment was aimed toward palliative care. The patient passed away three hours later. This case illustrates the acute and lethal nature of ACS in a less studied population, the pediatric oncologic patient. Prompt detection and treatment of ACS are essential for the management of critically ill pediatric patients, especially in those with space occupying tumors within the abdominal cavity. However, extreme presentations of ACS can have lethal consequences despite swift surgical intervention and adequate management.
RESUMO
Solitary fibrous tumors (SFTs) are rare soft tissue tumors that can arise in the abdomen, pleura, and central nervous system, among other sites. Surgical resection is the mainstay of management, although recurrence rates remain substantial. This case describes a 73-year-old male treated surgically for both a recurrent SFT and small bowel obstruction (SBO) secondary to adhesions. The patient had undergone numerous intra-abdominal operations for malignant SFT since 1994, highlighting the importance of meticulous resection at the initial presentation of local disease.
RESUMO
Inflammatory myofibroblastic tumors (IMTs) are rare mesenchymal neoplasms containing spindle cells and inflammatory components that can be locally aggressive. They have unclear biological behavior and may recur after resection. A 31-year-old woman presented with three months of cough, fatigue, weight loss, abdominal pain, anemia, and elevated inflammatory markers. CT showed a large well-circumscribed enhancing mass in the right colic mesentery. The patient underwent a laparoscopic right colectomy. Pathologic review showed fascicular spindle cells with admixed chronic inflammatory cells. Cells stained diffusely positive for SMA and anaplastic lymphoma kinase (ALK), diagnostic of an IMT. Post-operatively, the patient reported symptom resolution and had normalization of lab values. She remains disease-free at 20 months. IMT is rare in adults, accounting for 0.7%-1.0% of lung tumors. Up to 30% of patients present with elevated inflammatory markers. On imaging, IMTs are soft tissue masses with variable enhancement and fibrosis, often suspected to be malignant neoplasms. Up to 80% of IMTs are driven by altered tyrosine kinase signaling and half of IMTs express ALK, which may be treated in unresectable/recurrent cases using ALK-inhibitors. IMT may recur in 10%-15% of patients. The roles of adjuvant treatments are unclear given the rarity and unpredictable biological behavior. Long-term follow-up with regular radiologic and laboratory surveillance is recommended given possible local recurrence. IMTs are best managed in a multidisciplinary setting given their unpredictable nature. Surgery is the mainstay of IMT treatment with long-term control expected in >80% of adult patients.