Tech Coloproctol (2009) 13:79–81 DOI 10.1007/s10151-008-0430-x
C A S E R E P O RT
Pelvic malakoplakia simulating recurrence of rectal adenocarcinoma: report of a case S.F. Rafailidis • K.D. Ballas • N. Symeonidis • T.E. Pavlidis • E. Emoniotou • K. Psarras • A. Pantzaki • G.N. Marakis • A.K. Sakadamis
Received: 26 July 2007 / Accepted: 23 May 2008 / Published online: 5 August 2008 © Springer-Verlag 2008
Abstract Malakoplakia represents a chronic inflammatory disease associated with a broad spectrum of bacterial infections. On histopathology, Michaelis-Gutmann bodies are considered as pathognomonic histopathological findings. Rarity of the disease and wide variety of clinical presentation makes its diagnosis very challenging. We report herein the case of a 66-year-old woman who, having undergone lower anterior resection for rectal adenocarcinoma 3 and a half years ago, presented with urinary frequency and dull abdominal pain. CT scan revealed a soft tissue tumour infiltrating the preperitoneal fat over the urinary bladder, which was considered as recurrence of the rectal carcinoma. On laparotomy, a tumour invading the bladder, small intestine and the anterior abdominal wall was resected and the patient recovered uneventfully. On histopathology, malakoplakia of urinary bladder
was revealed. Overstaging of patients’ malignancy due to malakoplakia is not uncommon, so its early identification can help avoid incorrect treatment. Key words Malakoplakia · Colorectal carcinoma · Macrophage dysfunction · Tumour overstaging
Introduction Malakoplakia is an uncommon chronic granulomatous process that most commonly affects the urinary tract but the involvement of other organs has also been reported. It appears to result from a defect in macrophage function resulting in an inability to destroy ingested bacteria. The rarity of the disease and wide variety of clinical presentations makes its diagnosis very challenging.
S.F. Rafailidis · K.D. Ballas · N. Symeonidis · T.E. Pavlidis · E. Emoniotou · K. Psarras · G.N. Marakis · A.K. Sakadamis 2nd Propedeutical Department of Surgery Aristotles University of Thessaloniki, Greece A. Pantzaki Department of Pathology Ippokration General Hospital Thessaloniki, Greece S.F. Rafailidis (쾷) Kassandria Halkidikis, 63077 Kassandria, Greece e-mail: [email protected]
A 66-year-old female patient complained of a dull pain on the hypogastrium along with consistent urinary frequency and constipation of 4 weeks duration. She had a history of rectal adenocarcinoma, treated with low anterior rectosigmoidectomy 3.5 years earlier, and no previous history of urinary problems. She had an elevated white blood cell count and urinalysis showed many white and red blood cells. Coloscopy was normal and a CT scan showed a pelvic soft-tissue mass infiltrating the rectus abdominis muscles and the preperitoneal fat over the urinary bladder (Fig. 1). Recurrence of rectal adenocarcinoma was considered obvious and the patient underwent laparotomy. No evidence of local recurrence or generalized carcinoma was revealed and the tumour mass, pre-
Fig. 1 CT image reveals the presence of a soft-tissue mass infiltrating the preperitoneal fat over the urinary bladder
Fig. 2 Histological section reveals histiocytes containing characteristic inclusions of Michaelis-Gutmann type (PAS stain, ×400)
operatively depicted by the CT scan, measured approximately 15 cm in its greater dimension and infiltrated the rectus abdominis muscles, small intestine loops and the fundus of the urinary bladder. The lesion was resected en bloc along with part of the urinary bladder, small intestine and part of the anterior abdominal wall. The patient recovered uneventfully and left hospital on the 10th postoperative day. Histopathological examination showed widespread ulcerations of the mucosa of the urinary bladder and inflammatory infiltration of the bladder wall by histiocytes containing inclusions of the MichaelisGutmann type, findings characteristic of malakoplakia of the urinary bladder (Fig. 2).
Discussion Malakoplakia is a rare entity with approximately 450 cases reported since it was first described . It pre-
Tech Coloproctol (2009) 13:79–81
dominantly occurs in the genitourinary tract (60%) and less frequently in the gastrointestinal tract (10%), while it has been reported in the skin, brain, bone, thyroid and parotid gland . Extensive pelvic and abdominal wall involvement, as noted in our report, has been previously recognized in a very few cases . Although the pathogenesis of malakoplakia is not completely understood, it can be considered as the result of impairment of the mononuclear phagocyte and immunoregulatory system . Intracellular killing and digestion of bacteria within phagolysosomes is defective, resulting in degenerative changes and deposition of calcium and iron, thus forming the characteristic MichaelisGutmann bodies . Immunosuppressive therapy given for associated conditions may play a role in facilitating the proliferation of bacteria  and nearly 40% of patients with malakoplakia have been found to have primary or acquired immunodeficiency . The majority of bacteria associated with malakoplakia are coliforms and especially Escherichia coli, but other microorganisms have also been identified including Aerobacter, Klebsiella, Enterobacter, Proteus and Pseudomonas . Clinical presentation, as well as treatment of malakoplakia, depends variably on the organ involved. Urinary tract involvement, which is the most common, presents with haematuria and recurrent infections. When the lesion is large enough, obstructive symptoms can occur, and genital or gastrointestinal malakoplakia can present with vaginal or rectal bleeding, respectively . Adequate treatment includes a combination of surgery, antibiotics and cessation or decrease of immunosuppressive therapy [6, 7]. Chang and Kwok  reported 36 cases of colonic malakoplakia, while a MEDLINE search by Matter et al. revealed a total of 20 cases of malakoplakia with associated colorectal tumours . Malakoplakia can mimic colorectal malignancy or, as in our patient, simulate recurrence. This may lead to preoperative overstaging in patients with advanced rectal cancer and unnecessary neoadjuvant chemoradiation therapy . The surgeon must be aware of the disease and should obtain tissue for intraoperative frozen sections, when in doubt. Diagnosis though is not always possible as inflammation and malignancy can coexist, as in the case of malakoplakia and colorectal cancer . Furthermore, extended pelvic lesions can simulate advanced disease, which have no chance of cure leading to palliative therapy. In conclusion, although rare, malakoplakia should be considered in the differential diagnosis of lesions involving the genitourinary and gastrointestinal systems, especially in the immunosuppressed patient. Since malako-
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plakia can coexist with malignancy, maintaining a high index of suspicion and its identification are important in order to avoid preoperative overstaging or inappropriate palliation therapy. Conflict of interest statement The authors declare that they has no conflict of interest related to the publication of this article.
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