1 Ultrasound Obstet Gynecol 2011; 37: Published online in Wiley Online Library (wileyonlinelibrary.com). Letters to the Editor Subserosal pyomyoma in a virgin female: sonographic and computed tomographic imaging features A 42-year-old sexually inactive woman presented to our emergency department with worsening abdominal pain and high fever of 1 day s duration. During the previous month, she had had occasional mild lower abdominal pain, but without other symptoms. Her past medical history was unremarkable except for a myomectomy 10 years previously. Physical examination revealed diffuse abdominal tenderness with rebound. Laboratory data were notable for a leukocyte count of per mm 3 with a left shift. Pelvic sonography showed a large unilocular cystic structure in the upper pelvis measuring cm that demonstrated internal debris and a hyperechoic thick wall (Figure 1a). Contrast-enhanced computed tomography revealed a large cystic mass with a thick wall, which was posterior to and superior to the uterus (Figures 2a and c). Bilateral ovarian cysts were also noted (Figure 2b). Even in the absence of risk factors for ascending genital-tract infection, tubo-ovarian abscess was still the primary consideration based on the laboratory results and imaging findings. Antibiotic therapy was started immediately, but the patient s symptoms persisted. The following day an exploratory laparotomy revealed purulent material in the peritoneal cavity and a thick-walled pelvic mass containing purulent material. The mass was found to originate from the posterior wall of the uterus. Myomectomy could not be performed because the mass had severe adhesions. Instead, a tumor incision, marsupialization and drainage were performed. Microscopically, the mass consisted of fibrous tissue and smooth muscle cells, along with granulation tissue and chronic inflammation. No evidence of malignancy was noted. The patient had an uneventful recovery and was discharged within a week after surgery. Pelvic ultrasound 4 months after surgery showed that the size of the mass had decreased to 3 cm (Figure 1b). She remained well at the 12-month follow-up visit. Pyomyoma, or a suppurative leiomyoma, is a rare and potentially fatal complication of uterine leiomyoma. Only 21 cases have been reported since Most cases occur during pregnancy or after menopause, and are caused by ascending genitaltract infection. To our knowledge, this is the first case of pyomyoma described in a healthy premenopausal female and unrelated to any focus of infection. Figure 1 (a) Preoperative ultrasound image showing a large unilocular cystic structure in the upper pelvis that demonstrated internal debris and a hyperechoic thick wall. (b) Pelvic ultrasound image 4 months after surgery showed that the size of the subserosal fibroid had decreased to 3 cm. M, tumor mass; U, uterus. Only surgical treatment is life saving for patients with pyomyoma 2,3. Clinical suspicion and recognition of the sonographic features may prompt surgical intervention; however, published case reports have described only nonspecific sonographic findings, which are not diagnostic. Most reports mention an enlarging heterogeneous pelvic mass with solid and cystic components 3 6. Nguyen and Gruenewald 9 stated that the presence of gas in uterine leiomyoma is highly suggestive of pyomyoma. In the present case the diagnosis was delayed because the patient had never had sexual activity and her subserosal pyomyoma presented as a large unilocular cystic mass resembling a tubo-ovarian abscess. In conclusion, the diagnosis of pyomyoma is difficult because of its diverse presentation and lack of characteristic imaging features. Thus, we suggest that in patients with a thick-walled pelvic mass and unexplained fever Copyright 2011 ISUOG. Published by John Wiley & Sons, Ltd. L E TTERS TO THE E D ITOR
2 248 Letters to the Editor Benign cystic peritoneal mesothelioma incorrectly diagnosed as an ovarian borderline mucinous tumor of intestinal type at transvaginal preoperative ultrasound evaluation Figure 2 Computed tomography images showing a thick-walled, 9-cm cystic mass within the upper pelvis, superior to the uterus. (a) Coronal view: white arrows indicate the cystic mass, black arrow indicates the uterus; (b) coronal view: arrows indicate bilateral ovarian cysts; (c) axial view of the pelvic tumor. or abdominal pain, pyomyoma should be included in the differential diagnosis. H.-S. Liu and C.-H. Chen* Department of Obstetrics and Gynecology, National Taiwan University Hospital and College of Medicine, National Taiwan University, Taipei, Taiwan ( DOI: /uog Prahlow JA, Cappellari JO, Washburn SA. Uterine pyomyoma as a complication of pregnancy in an intravenous drug user. South Med J 1996; 89: Genta PR, Dias ML, Janiszewski TA, Carvalho JP, Arai MH, Meireles LP. Streptococcus agalactiae endocarditis and giant pyomyoma simulating ovarian cancer. South Med J 2001; 94: Grune B, Zikulnig E, Gembruch U. Sepsis in second trimester of pregnancy due to an infected myoma. A case report and a review of the literature. Fetal Diagn Ther 2001; 16: Lin YH, Hwang JL, Huang LW, Chen HJ. Pyomyoma after a cesarean section. Acta Obstet Gynecol Scand 2002; 81: Karcaaltincaba M, Sudakoff GS. CT of a ruptured pyomyoma. AJR Am J Roentgenol 2003; 181: Mason TC, Adair J, Lee YC. Postpartum pyomyoma. JNatl Med Assoc 2005; 97: Calleja-Agius J, O Brien P, Iskaros J, Calleja N. Pyomyoma. J Obstet Gynaecol 2006; 26: Manchana T, Sirisabya N, Triratanachat S, Niruthisard S, Tannirandorn Y. Pyomyoma in a perimenopausal woman with intrauterine device. Gynecol Obstet Invest 2007; 63: Nguyen QH, Gruenewald SM. Sonographic appearance of a postpartum pyomyoma with gas production. J Clin Ultrasound 2008; 36: Fletcher H, Gibson R, Williams N, Wharfe G, Nicholson A, Soares D. A woman with diabetes presenting with pyomyoma and treated with subtotal hysterectomy: a case report. JMed Case Rep 2009; 3: An asymptomatic 70-year-old woman, gravida 3 para 3, was referred after an incidental finding of a left ovarian mass. At ultrasound examination a multilocular ovarian cyst, size mm, was diagnosed, with a very high number of locules and low-level cystic content. No solid component was identified within the cyst and the echogenic tissue was interpreted, at dynamic examination, as an aggregation of septa. The diameter of the locules ranged between 7 and 15 mm. At color Doppler examination, only a few vessels were observed within the septa. The ultrasound examiner entertained a diagnosis of mucinous borderline ovarian tumor of intestinal type, even if the small dimensions of the lesion were inconsistent with the normal size of this pathological entity (Figure 1a). The right ovary was reported as having a normal appearance. No free fluid in the pouch of Douglas was observed. Tumor markers (CA125, CA19-9) were negative. The patient s gynecological history was negative. The patient had previously undergone a cholecystectomy by laparotomy in 1983, and a left nephrectomy for a well-differentiated papilliferous adenocarcinoma in The pelvic findings were inconclusive. An open laparoscopy revealed a normal uterus and normal tubes. The left ovary was reduced in size, with a smooth surface, completely covered by innumerable small translucent vesicles tenaciously adherent to the lateral pelvic wall, the posterior leaf of the broad ligament, the pouch of Douglas, and the left side of the rectal wall (Figure 1b). The right ovary was also reduced in size, mobile and with 80% of its surface covered by the same soft translucent gelatinous vesicles (Figure 1c). The liver was not visible due to dense adhesions of the omentum to the anterior abdominal wall (caused by the previous abdominal surgery). There were no vegetations suspicious for malignancy on the pelvic and abdominal peritoneum, omentum and bowel serosa. A bilateral adnexectomy was performed using bipolar forceps and scissors, and the adnexa were removed from the abdominal cavity through an endoscopic bag. Attention was then focused on the peritoneal disease, and about 90% of the vesicles were removed. Since frozen section reported benign mesothelial cysts with no pathology of the adnexa, it was decided not to proceed with radical surgery, leaving fewer than 10% of vesicles tenaciously adherent to the peritoneum overlying the course of the great vessels. Final pathology confirmed a benign mesothelioma (Figure 1d), with no pathology of the adnexa, or of the multiple biopsy specimens (lateral pelvic walls, Douglas peritoneum, vesicouterine fold peritoneum or omentum). The morphological diagnosis was confirmed by immunohistochemical staining positive for calretinin, podoplanin, cytokeratin 5/6 and negative for carcinoembryonic antigen and BerEP4.
3 Letters to the Editor 249 Figure 1 (a) Ultrasound image of the left multilocular ovarian cyst. At color Doppler examination scarce vascularization was observed within the septa. The presence of a multilocular cyst suggested a diagnosis of a mucinous adnexal mass, suspicious for borderline tumor. (b) Laparoscopic findings showing innumerable small, thin-walled, translucent cysts completely covering the left ovary and tenaciously adherent to other structures. (c) The right ovary had 80% of its surface covered by the same cysts. (d) Microscopically the cysts were characterized by a single layer of flattened, benign-appearing mesothelial cells. The septa and walls contained considerable amounts of fibrous tissue and consisted of a loose, fibrovascular connective tissue with a sparse inflammatory infiltrate (hematoxylin eosin, original magnification, 100). Benign peritoneal cystic mesothelioma is an extremely rare tumor of unknown etiopathogenesis. No more than 140 cases had been reported up to These lesions occur predominantly in women of reproductive age, with a 5 : 1 female : male ratio. They typically present in the form of multiple, small, thin-walled, translucent cysts that have a smooth lining. Unlike malignant mesothelioma, benign cystic mesothelioma is not associated with prior exposure to asbestos. Most patients have a history of previous abdominal or pelvic surgery, endometriosis or pelvic inflammatory disease, suggesting a role for inflammation in the pathogenesis of the cyst. Some authors prefer the designation multilocular peritoneal inclusion cyst to benign cystic mesothelioma for such lesions, until there is convincing evidence for their neoplastic origin 2. Abdominal pain, tenderness and an abdominal or pelvic mass may be present, depending on the size of the tumor, but some patients may be asymptomatic. Prior studies have suggested that cure is only accomplished with surgical resection; however, patients have a 30 50% risk of recurrence, from 1 month to many years postoperatively. There is no correlation of risk of recurrence with extent of resection 3. This finding, together with the indolent course of the disease in many patients, makes less aggressive surgery attractive in the treatment of the benign peritoneal cystic mesothelioma. Therefore, minimizing the extent of surgery and the number of laparotomies may be appropriate when a
4 250 Letters to the Editor correct diagnosis of benign lesion can be made 4.There is only one case report of a benign to malignant transformation of peritoneal cystic mesothelioma 5.Thisfact mandates systematic clinical follow-up of these patients for prolonged periods, perhaps for life. Preoperative diagnosis of mesothelioma is challenging and scarce data are reported in the literature regarding the sonographic appearance of the disease 6,7. In the present case, an incorrect diagnosis of mucinous borderline tumor of intestinal type was made. Primary peritoneal serous tumor and carcinomatosis were not taken into consideration in the differential diagnosis, as no solid nodules were observed. In the presence of multilocular tissue located within the pelvis, the absence of a regular capsule and the absence of an ovoidal shape of the whole lesion should raise the suspicion of mesothelioma. A. C. Testa, G. F. Zannoni, S. Ferrari, A. Lecca, E. Marana and R. Marana* Department of Obstetrics and Gynecology, Catholic University of the Sacred Heart, Rome, Italy; Department of Pathology, Catholic University of the Sacred Heart, Rome, Italy; Department of Anesthesiology, Intensive Care and Emergency Medicine, Catholic University of the Sacred Heart, Rome, Italy ( DOI: /uog Machlenkin S, Diment J, Kashtan H. Benign cystic mesothelioma of the peritoneum. Isr Med Assoc J 2006; 8: Kurman RJ (ed.). Blanstein s Pathology of the Female Genital Tract (5 th edn). Springer-Verlag: New York, 2002; Vallerie AM, Lerner JP, Wright JD, Baxi LV. Peritoneal inclusion cysts: a review. Obstet Gynecol Surv 2009; 64: Søreide JA, Søreide K, Körner H, Søiland H, Greve OJ, Gudlaugsson E. Benign peritoneal cystic mesothelioma. World J Surg 2006; 30: González-Moreno S, Yan H, Alcorn KW, Sugarbaker PH. Malignant transformation of benign cystic mesothelioma of the peritoneum. J Surg Oncol 2002; 79: Bui-Mansfield LT, Kim-Ahn G, O Bryant LK. Multicystic mesothelioma of the peritoneum. AJR Am J Roentgenol 2002; 178: Jerbi M, Hidar S, Ziadi S, Khairi H. Benign multicystic peritoneal mesothelioma. Int J Gynaecol Obstet 2006; 93: Chiari malformation eponym time for historical justice There is a historical injustice surrounding the Chiari malformation eponym. Hans Chiari ( ) served as a pathology professor in Prague, where he described the alterations in the cerebellum resulting from cerebral hydrocephalus, later known as Chiari malformation. This is in part why I was disappointed to hear in that very same beautiful city, in multiple sessions at the 2010 International Society of Ultrasound in Obstetrics and Table 1 Results of PubMed search for Chiari malformation and Arnold Chiari malformation Language Number (%) of publications using the term: Chiari malformation Arnold Chiari malformation English 736 (71.8) 289 (28.2) German 2 (10.5) 17 (89.5) Other 75 (49.0) 78 (51.0) Gynecology (ISUOG) annual meeting, that chairs and speakers still use the incorrect eponym, Arnold Chiari malformation. Upon returning from Prague, on 17 October , I performed a PubMed search of publication titles for: Chiari malformation and Arnold Chiari malformation (Table 1). It is of interest that the highest rate of usage of the incorrect term is in the German language literature, the native language of both Chiari and Arnold. Following these results I decided to try and bring to the attention of the White Journal readers the historical injustice surrounding this eponym. In 1883, the British anatomist John Cleland ( ) was the first to publish a brief description of an infant with spina bifida and hydrocephalus 1. In 1891 and 1895, Chiari published his extensive and thorough landmark descriptions 2,3, which contained comprehensive neuropathological evaluations of the malformation. In 1894, Julius Arnold ( ) published a case report of an infant with a sacral teratoma, but without hydrocephalus, and described a ribbon of tissue that protruded through the foramen magnum 4. Arnold did not include in his report a pathological evaluation or further information, and did not cite the previous works of Cleland and Chiari, while Chiari had cited Cleland 1 in his first publication 2, and went on to cite both Cleland 1 and Arnold 4 in his second publication 3. Both Chiari and Arnold published their reports in German and it is highly unlikely that Arnold was unaware of Chiari s first publication. Earlier descriptions 5, and the observations of both Cleland and Arnold, were incidental. The erroneous eponym Arnold Chiari malformation was coined in 1907 by Schwalbe and Gredig 6, two students who worked in Arnold s laboratory in Heidelberg, and has since then been used widely. This historical injustice has been corrected 7 10, but unfortunately people still use the incorrect term. I hope that this letter will convince the medical community to favor the correct eponym: Chiari malformation. I. Solt Maternal-Fetal Medicine Division, Cedars Sinai Medical Center, Los Angeles, CA, USA ( DOI: /uog.8876
5 Letters to the Editor Cleland J. Contribution to the study of spina bifida, encephalocele, and anencephalus. J Anat Physiol 1883; 17: Chiari H. Ueber Veränderungen des kleinhirns infolge von hydrocephalie des grosshirns. Dtsch Med Wochenschr 1891; 17: Chiari H. Ueber Veränderungen des kleinhirns, des pons, und der medulla oblungata infolge von congenitaler hydrocephalie des grosshirns. Denkschr Akad Wissensch Math Naturw Cl 1895; 63: Arnold J. Myelocyste, transposition von gewebskeimen und sympodie. Beilr Pathol Anat 1894; 16: Cruveilhier J. L Anatomie Pathologique du Corps Humain; Descriptions avec Figures Lithographiées et Coloriées; Diverses Alterations Morbides don t le Corps Humain et Susceptible (2 vols). Bailliere: Paris, Schwalbe E, Gredig M. Ueber entwicklungsstorungen des kleinhirns, hirnstamms und halsmarks bei spina bifida (Arnold sche und Chiari sche missbildung). Beitr Pathol Anat 1907; 40: Pearce JMS. Arnold chiari, or Cruveilhier Cleland Chiari malformation. J Neurol Neurosurg Psychiatry 2000; 68: Arnett B. Arnold-Chiari malformation. Arch Neurol 2003; 60: Sarnat HB. Semantics do matter! Precision in scientific communication in pediatric neurology. J Child Neurol 2007; 22: Bejjani GK. Definition of the adult Chiari malformation: a brief historical overview. Neurosurg Focus 2001; 11: 1 8.