Patient: Female, 31-year-old
Final Diagnosis: Appendiceal neuroendocrine tumor • low-grade appendiceal mucinous neoplasm
Symptoms: Abdominal pain • dysuria
Medication: —
Clinical Procedure: Laparoscopic appendicectomy • laparoscopic right hemicolectomy
Specialty: Oncology • Surgery
Objective:
Rare co-existance of disease or pathology
Background:
Incidental appendiceal neoplasms account for 1–2% of appendectomies. Mucinous neoplasms and carcinoids are the most frequent lesions, with an incidence of 0.6% and 0.3–0.9%, respectively. Appendiceal collision tumors are extremely rare and result from the proliferation of 2 different cellular lines. This report describes a young woman with a collision tumor composed of a low-grade appendiceal mucinous neoplasia (LAMN) and an appendiceal neuroendocrine tumor (ANET).
Case Report:
A 31-year-old woman was admitted to our institution presenting with abdominal pain and dysuria. After ultrasound assessment of a dilated appendix with wall thickening and distension by anechogenic material, a diagnosis of acute appendicitis was made. The patient, after a period of antibiotic therapy and observation, underwent an urgent laparoscopic appendectomy due to worsening condition. Surprisingly, the histological exam revealed a Tis LAMN extending from the base of the appendix to the resection margins, and a T3 grade-1 ANET, chromogranin-A and synaptophysin-positive, with a Ki67 less than 1%. On the basis of histological examination and European Neuroendocrine Tumor Network guidelines, in light of the positive LAMN resection margin and ANET mesoappendiceal invasion, after multidisciplinary team discussion, an elective laparoscopic hemicolectomy was indicated. The patient is now in good condition following a regular 5-year follow-up.
Conclusions:
A collision LAMN and ANET is an exceedingly rare condition. The heterogeneity of clinical presentation and lack of solid evidence seem to recommend a tailored management. Laparoscopy is a safe and useful tool in localized mass excision.
Primary cutaneous CD30+ lymphoproliferative disorders include primary cutaneous anaplastic large cell lymphoma (pcALCL) and lymphomatoid papulosis. The prognosis of the disease is usually excellent but, in a minority of cases, it presents with extracutaneous involvement and aggressive behavior. The case we present—relapsed after surgical excision, immunosuppressive therapy, and conventional chemotherapy—is the first one treated with Autologous Stem Cell transplant followed by Brentuximab Vedotin consolidation, a scheme already used for high risk Hodgkin Lymphoma.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.