title: Perforated appendiceal diverticulitis associated with appendiceal neurofibroma in neurofibromatosis type 1.
abstract: An appendiceal neurofibroma (ANF) is a rare neoplasm associated with neurofibromatosis type 1(NF-1), an inheritable neurocutaneous disorder that involves multiple systems including the intraabdominal organs. Appendiceal diverticulitis occasionally ruptures in the absence of intense abdominal pain, which can lead to serious consequences. Recent reports highlight the association between appendiceal diverticulum and appendiceal neoplasms; however, there is still little information on the association between appendiceal diverticulitis and ANF in NF-1. A 51-year-old Japanese male with NF-1 was referred to the division of surgery for mild right lower quadrant pain. It was suspected he had perforated acute appendicitis with periappendiceal abscess based on clinical manifestations and findings of computed tomography. An emergency appendectomy was conducted. The pathological examination revealed diffusely proliferated tumor cells of a neurofibroma, coexistent with multiple appendiceal diverticulums, leading to the diagnosis of perforated appendiceal diverticulitis associated with ANF. Although he developed a remnant abscess, he recovered with the conservative treatments of antibiotics and drainage. This case suggests that appendiceal diverticulitis might be a complication of appendiceal involvement of NF-1, and that it occasionally ruptures in the absence of intense abdominal pain. Clinicians should recognize that NF-1 can cause various abdominal manifestations.
authors.affiliation: Akihiko Ozaki, Manabu Tsukada, Hiromichi Ohira, Division of Surgery, Minamisoma Municipal General Hospital, Fukushima 975-0033, Japan.
authors.forename: Akihiko
authors.initials: A
authors.lastname: Ozaki
authors.affiliation: Akihiko Ozaki, Manabu Tsukada, Hiromichi Ohira, Division of Surgery, Minamisoma Municipal General Hospital, Fukushima 975-0033, Japan.
authors.forename: Manabu
authors.initials: M
authors.lastname: Tsukada
authors.affiliation: Akihiko Ozaki, Manabu Tsukada, Hiromichi Ohira, Division of Surgery, Minamisoma Municipal General Hospital, Fukushima 975-0033, Japan.
authors.forename: Kazuo
authors.initials: K
authors.lastname: Watanabe
authors.affiliation: Akihiko Ozaki, Manabu Tsukada, Hiromichi Ohira, Division of Surgery, Minamisoma Municipal General Hospital, Fukushima 975-0033, Japan.
authors.forename: Masaharu
authors.initials: M
authors.lastname: Tsubokura
authors.affiliation: Akihiko Ozaki, Manabu Tsukada, Hiromichi Ohira, Division of Surgery, Minamisoma Municipal General Hospital, Fukushima 975-0033, Japan.
authors.forename: Shigeaki
authors.initials: S
authors.lastname: Kato
authors.affiliation: Akihiko Ozaki, Manabu Tsukada, Hiromichi Ohira, Division of Surgery, Minamisoma Municipal General Hospital, Fukushima 975-0033, Japan.
authors.forename: Tetsuya
authors.initials: T
authors.lastname: Tanimoto
authors.affiliation: Akihiko Ozaki, Manabu Tsukada, Hiromichi Ohira, Division of Surgery, Minamisoma Municipal General Hospital, Fukushima 975-0033, Japan.
authors.forename: Masahiro
authors.initials: M
authors.lastname: Kami
authors.affiliation: Akihiko Ozaki, Manabu Tsukada, Hiromichi Ohira, Division of Surgery, Minamisoma Municipal General Hospital, Fukushima 975-0033, Japan.
authors.forename: Hiromichi
authors.initials: H
authors.lastname: Ohira
authors.affiliation: Akihiko Ozaki, Manabu Tsukada, Hiromichi Ohira, Division of Surgery, Minamisoma Municipal General Hospital, Fukushima 975-0033, Japan.
authors.forename: Yukio
authors.initials: Y
authors.lastname: Kanazawa
mesh_terms: D015746:Abdominal Pain / Q000209:etiology; D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D001062:Appendectomy; D001063:Appendiceal Neoplasms / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D001706:Biopsy; D004238:Diverticulitis / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D004322:Drainage; D006801:Humans; D008297:Male; D008875:Middle Aged; D009456:Neurofibromatosis 1 / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D012422:Rupture, Spontaneous; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
chemical_list: D000900:Anti-Bacterial Agents
keywords: Appendiceal diverticulitis; Appendiceal diverticulum; Appendiceal neurofibroma; Appendiceal perforation; Neurofibromatosis type 1