後腹膜に原発し, vonRecklinghausen氏病の徴候のない孤立性神経線維腫の1例を報告する.診断上, もう一つの神経鞘由来の神経鞘腫との鑑別が問題で, 腫瘍の組織化学染色および免疫組織化学染色が孤立性神経線維腫の診断に有用であった,A solitary neurofibroma arising in the retroperitoneal space without any other stagma of von Recklinghausen's disease is reported. Confusion with another nerve sheath tumor, a schwannoma is a diagnostic pitfall. Histochemical and immunohistochemical stainings of the tumor are useful for the diagnosis of solitary neurofibroma

HARA, Isao

HARA, Shinji

ISHIKAWA, Jiro

KAMIDONO, Sadao

MAEDA, Sakan

OHMAE, Hiroshi

SUGIYAMA, Taketoshi

TAKECHI, Yoshizumi

Kyoto University Research Information Repository

Title Solitary retroperitoneal neurofibroma: a case reportAuthor(s)ISHIKAWA, Jiro; KAMIDONO, Sadao; MAEDA, Sakan;SUGIYAMA, Taketoshi; HARA, Isao; TAKECHI, Yoshizumi;OHMAE, Hiroshi; HARA, ShinjiCitation泌尿器科紀要 (1989), 35(7): 1157-1160Issue Date1989-07URL http://hdl.handle.net/2433/116604RightType Departmental Bulletin PaperTextversionpublisherKyoto UniversityActa Urol. Jpn. 35: 1157-1160, 1989 1157 SOLITARY RETROPERITONEAL NEUROFIBROMA: A CASE REPORT Jiro ISHIKAWA and Sadao KAMIDONO From the Department oj Urology, Kobe University School oj Medicine Sakan MAEDA and Taketoshi SUGIYAMA From the Department oj Pathology, Kobe University School oj Medicine Isao HARA, Y oshizumi T AKECHI, Hiroshi OHMAE and Shinji HARA From Hara Genitourinary Hospital A solitary neurofibroma arising in the retroperitoneal space without any other stagma of von Recklinghausen's disease is reported. Confusion with another nerve sheath tumor, a schwannoma is a diagnostic pitfall. Histochemical and immunohistochemical stainings of the tumor are useful for the diagnosis of solitary neurofibroma. Key words: Solitary neurofibroma, Retroperitoneal space, Alcian blue staining, S-100, NSE INTRODUCTION A neurofibroma is a benign nerve sheath tumor that occurs as a solitary tumor or as a partial manifestation of von Reckling-hausen's disease: an inheritant disease characterized by multiple cutaneous pig-mented lesions (cafe-au-lait spots) and subcutaneous neurofibromas. Although genitourinary involvement of neurofibro-mas is rare, such cases have been reported in the bladder, kidney, ureter, penis, and retroperitoneal space l- 6) Previous lit-erature shows confusion relating to the terminology of the peripheral nerve tumors7). We report a case of a solitary retroperitoneal neurofibroma diagnosed by histochemical and immunohistochemical stainings as well as light microscopic ap-pearance of the tumor. CASE REPORT A 56-year-old Japanese woman presented with a 2-year history of dysuria which fluctuated with periods of exacerbations and remissions. Significant medical history included total abdominal hysterectomy for leiomyomas 22 years earlier. Family his-tory was negative for von Recklinghaus-en's disease. Physical examination reveal-ed traction pam m the left lower limb. Cutaneous examination revealed no cafe-au-lait spots or neurofibromas. All the hematological and serum biochemical data, and urinalysis revealed no abnormal-ity. Drip infusion pyelogram (DIP) dem-onstrated the medially dislocated bladder and the left ureter, suggesting the existence of the extravesical mass (Fig. 1). On cystoscopy, the bladder mucosa was intact. Computerized tomography (CT) demon-strated a well encapsulated homogeneous mass in the retrovesical space (Fig. 2). Exploratory laparotomy, performed on May 2 1987, revealed an elastic soft tumor in the retrovesical space. The 8 x 8.5 x 8 cm tumor, weighing 326 g was completely excised. Cut surface of the tumor was yellowish-gray in color, and gela tinous. Microscopic examination of the excised tumor revealed fusiform tumor cells in numerous collagen fibrils and a non-oraganized matrix (Fig. 3). The cell arrangement of Antoni type A and type B was not observed. No cellular atypism and mitotic figures were observed. Alcain blue staining showed positive findings in the matrix. Immunohistostaining for S-100 protein and neuron specific enolase (NSE) showed positive in the tumor cells 1158 Acta Uro!' Jpn. Vol. 35 No. 7, 1989 Fi g . I. DIP shows the di splace ment of the bladder and le ft u re ter medially, sug-gesting a large extravesical m ass. Fig. 2. CT demonstr ates a r e trovesical m ass. Fig. 3. Histolog ic appearance of the tumor shows fusiform tu mor ce ll s in a m atrix (H & E stain ). (Fig. 4, A and B) . Thus, the tumor was diagnosed as a solitary neurofibroma . The patient remains well without any signs of tumor recurrence. DISCUSSION A neurofibroma and a schwannoma (, \ .,,' Fi g. 4. Immunohistochemical demontsration of S-IOO (A ) and NSE (B ). The tumor cells show an intense positive immu-noreaction in the cytoplasm ( PAP im-mun operoxidase stain without co unter sta in ). (neurilemoma) are benign nerve sheath tumors, originating from the Schwann cells. Although there is confusion relating to differentiation between a neurofibroma and a schwannoma, a neurofibroma is distinct from a schwannoma in the follow-ing points . (i) The former lacks the compact arrangement of cells m the Antoni type A tissue and delicate single cell thick fascicles of the Antoni type B tissue of a schwannoma i ) (ii) Alcian blue stains for acid mucopolysaccharides are negative in a schwannoma but positive in a neurofibroma7) , (iii ) A neurofibroma conta ins fewer S-IOO protein positive cells than a schwannomaE) , S-lOO, a dimetric calcium binding pro-tein, composed of different combination of (\' and ~ subunits, mainly locates in glial and Schwalm cells·), NSE, a glyco-lytic dimetric enzyme, locates in neurons and neuroendocrine cells10) , Immunohis-tochemical demonstration of S-IOO and NSE is valuable m the diagnosis of Ishikawa et al.: Retroperitoneal neurofibroma 1159 a nerve sheath tumor. Unbalanced expres-sion of S-100 subunits is observed in malignant nerve sheath tumors. Hayashi et al. observed an increased expression of the S-100 IX subunit in the malgnant part of neurofibromasll). They concluded that the S-100 subunit immunoreactivity is a good marker for malignant schwannomas. As described above, we could not distinguish between a neurofibroma and a schwannoma in the previous literature. Das Gupta et al. considered solitary neu-rofibromas and schwannomas (neurilemo-mas) as the same entity, and they re-viewed 303 such cases!2). In that report, most tumors occurred in the head and neck, and extremities. On the other hand, only two of these cases (0.66 %) were retroperitoneal origin. Surgical excision of the tumor is the choice of treatment6)· Careful search for the coexistence of a malignant tumor is necessary, because 16 % of the patients with a solitary neuro-fibroma were reported to have an associ-a ted malignan t tumor 12) • It remains to be elucidated whether the clinical course of a solitary neurofibroma d;ffers from that of a schwannoma. REFERENCES 1) Gonzalez-Angulo A and Reyers HA: Neuro-fibromatosis involving the lower urinary tract. J Urol 89 :804-811, 1963 2) Winfield HN and Catalona WJ: An isolated plexiform neurofibroma of the bladder. J Urol 134: 542543, 1985 3) Freund ME, Crocker DW and Harrisier JH Neurofibroma arising III a solitary kidney. J Urol 98: 318-321, 1967 4) Ravich A: Neurofibroma of the ureter: report of a case with operative and recovery. Arch Surg 30: 442-448, 1935 5) Elliott FG, Eid TC and Lakey WH: Gen-itourinary neurofibromatosis clinical significance. J Urol 125: 725-727, 1981 6) Carpenter WB and Kernohan JW: Retro-peritoneal ganglioncuromas and neurofibro-mas: a clinicicopathological study. Cancer 16: 788-797, 1963 7) Harkin JC and Reed RJ: Solitary neuro-fibroma (Solitary nerve sheath tumor). In : Tumors of the peripheral nervous system. Edited by Hasrkin JC and Reed RJ. Armed Forces Institute of Pathology, Fasc. 3, pp. 51-59, Washington, D.C., 1968 8) Weiss SW, Langloss JM and Enzinger FM: S-IOO protein in the diagnosis of soft tissue tumors with particular reference to benign and malignant Schwann cell tumors. Lab Invest 49: 299-308, 1973 9) Stefansson K, Wollmann RL and Moore BW Distribution of S-100 protein outside the central nervous system. Brain Res 234: 309-317, 1982 10) Kato K, Suzuki F and Semba R. Deter-mination of brain enolase isozymes with an enzyme immunoassay at the level of single neurons. J Neurochem 37: 998-1005, 1981 II) Hayashi K, Tak ahashi K, Sonobe H, Ohtsuki Y and Taguchi K :The distribution of alpha and beta subunits of S-IOO protein in malignant Schwannomas arising from neu-rofibromatosis of von Recklinghausen's disease. Virchows Arch A 411 : 515-521, 1987 12) Das Gupta TK, Brasfield RD, Stbong EW and Hajdu SI: Benign solitary Schwanno-mas (Neurolenomas). Cancer 24: 355-366, 1969 13) Steers WD, Hodge GB, Johnson DE, Chai-tin BA and Charasangavej C: Benign retro-peritoneal neurilemoma without von Reck-linghausen's disease: a rare occurrence. J Urol 133: 846-848, 1985 (Accepted for publication July 25, 1988) 1160 Acta Urol. Jpn. Vol. 35 No. 7, 1989和文抄録孤立性後腹膜神経線維腫の1例神戸大学医学部泌尿器科学教室(主任:守殿貞夫教授)石 川 二 朗,守 殿 貞 夫神戸大学医学部第2病理学教室(主任:杉山武敏教授)前 田 盛,杉 山 武 敏原泌尿器科病院(院長:原 信二)原 勲,武市 佳純,大 前 博志,原 信二後腹膜に原発し,vonRecklinghausen氏病の徴候のない孤立性神経線維腫の1例 を報告する.診 断上,も う一つの神経鞘由来の神経鞘腫 との鑑別が問題で,腫瘍の組織化学染色および免疫組織化学染色が孤立性神経線維腫の診断に有用であった,(泌尿紀要35:ll57-1160,1989)

孤立性後腹膜神経線維腫の1例

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孤立性後腹膜神経線維腫の1例

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