82歳女.主訴は排尿困難と残尿感.膀胱底部から尿道周囲にかけて鶏卵大の腫瘤を認め, 生検で非ホジキンリンパ腫と診断された.他臓器に病変を認めなかった為, 尿道原発の悪性リンパ腫と診断し放射線療法を施行した.治療後, 腫瘍は著明に縮小したが肺転移が出現した.エトポシド単剤による化学療法を追加したが, 放射線治療後5ヵ月で腫瘍の急激な増加を認め癌死したA patient with a primary malignant lymphoma surrounding the female urethra is reported. Despite the good response of the primary tumor to radiotherapy, the patient died shortly after diagnosis due to disseminated disease. We reviewed 16 cases of this rare entity reported previously

OGAWA, Osamu

OKADA, Yusaku

SHIMIZU, Yousuke

TERACHI, Toshiro

YOSHIDA, Osamu

Kyoto University Research Information Repository

TitleA case of primary urethral lymphoma presenting as a hugemass surrounding the female urethraAuthor(s)SHIMIZU, Yousuke; OGAWA, Osamu; TERACHI, Toshiro;OKADA, Yusaku; YOSHIDA, OsamuCitation泌尿器科紀要 (1997), 43(3): 229-232Issue Date1997-03URL http://hdl.handle.net/2433/115919RightType Departmental Bulletin PaperTextversionpublisherKyoto UniversityActa Urol. Jpn. 43: 229-232, 1997 229 A CASE OF PRIMARY URETHRAL LYMPHOMA PRESENTING AS A HUGE MASS SURROUNDING THE FEMALE URETHRA Yousuke SHIMIZU, Osamu OGAWA, Toshiro TERACHI, Yusaku OKADA and Osamu YOSHIDA From the Department oj Urology, Faculty oj Medicine, Kyoto University A patient with a primary malignant lymphoma surrounding the female urethra is reported. Despite the good response of the primary tumor to radiotherapy, the patient died shortly after diagnosis due to disseminated disease. We reviewed 16 cases of this rare entity reported previously. (Acta Urol. Jpn. 43: 229-232, 1997) Key words: Female urethra, Malignant lymphoma, Radiotherapy INTRODUCTION Malignant lymphoma can affect almost any organ of the body, although it usually originates in the lymph nodes. The incidence of malignant lym-phomas in urogenital organs is low. Limited clinical experience has resulted in uncertainty concerning the most appropriate form of therapy for this malignancy. We report an 82-year-old woman with non-Hodgkin's lymphoma presenting as a huge tumor surrounding the female urethra. CASE REPORT An 82-year-old woman consulted our hospital with complaints of dysuria and sense of residual urine Fig. I. MRI revealed a large homo-geneous mass (5.5 cm X 5 cm X 5.2 cm) surrounding the urethra. which had persisted for 2 months. Physical examination revealed an elastic hard mass along the urethra. Laboratory examination revealed no abnormal findings except the elevation of serum level of lactic dehydrogenase (555 IU!L; normal < 230). Cystoscopy revealed the bladder neck elevated by the mass around the urethra, although the bladder mucosa was smooth, indicating that the tumor had originated from the paraurethral region. Gyneco-logical examination did not reveal any abnormal findings in female genital organs. Pelvic computed tomography (CT) and magnetic resonance imaging (MRI) revealed a 5.5 cmX5 cmX5.2 cm mass surrounding the urethra (Fig. 1). Cold cup biopsy was performed, and the histopathological exami-nation showed infiltration of poorly differentiated malignant cells (Fig. 2). After immunohisto-chemical staining of the specimen (CD20 (+), CD45RO (-), CD3 (-), CD15 (-), CD68 (-)), the tumor was diagnosed as non-Hodgkin's lymphoma, B cell type, diffuse and large cell type according to the Lymphoma Study Group in Japan (LSG) classification J). There was no evidence of tumor elsewhere under radiological examination. The Fig. 2. Histological examination of the biopsy specimen revealed proliferation of small and large malignant cells that were diagnosed as lymphoma cells by immunohistochemical analysis. 230 Acta Urol. Jpn. Vol. 43, No. 3, 1997 bone marrow aspiration biopsy failed to show the infiltration of lymphoma cells. Under the diagnosis oflocally invasive urethral malignant lymphoma, the patient underwent 40 Gy radiotherapy to the whole pelvis and the bilateral inguinal region. Although the primary lesion responded well to the radio-Fig. 3. MRI after radiotherapy revealed a remarkable response in the primary tumor. Table I. Summary of reported Age Presentation Stage Unknown Unknown 83 Dysuria, Meatal mass Local 51 Urethral polyp Local 76 Large urethral mass Local 31 Spotting, Firm urethra Local 63 Urethral mass Local 78 Polyuria, Dysuria Local 65 Vaginal spotting Local 76 Caruncle, Hematuria Locally invasive 63 Vulvar pruritus, Dysuria Locally invasive 75 Vaginal spotting Locally invasive 62 Caruncle, Spotting Disseminated 53 Caruncle Disseminated 77 Vaginal mass, Hematuria Disseminated 57 Hematuria, Difficult voiding Disseminated 57 Urinary retention Disseminated 82 Dysuria Locally invasive therapy (Fig. 3), multiple lung metastases appeared during the therapy. The patient died of diss-eminated disease five months after radiotherapy despite oral administration of25 mg/day of etoposide. DISCUSSION According to Freeman's study2), primary uro-genital malignant lymphomas are relatively rare, and comprised 2.6% of all extranodal lymphomas. To the best of our knowledge, only 16 cases of urethral lymphoma, including 2 male patients, have been reported, all being histopathologically diagnosed as non-Hodgkin lymphomas (Table I). Urethral lymphomas usually present with a painless mass resembling a caruncle. This patient, however, had a huge mass beneath the bladder neck indicating infil tra tion to the periurethral tissue. This parti-cular pattern of tumor growth was the cause of her major complaint of dysuria with a sense of residual unne . There is no consensus about treatment of urethral lymphoma. Various treatments have been applied to urethral lymphoma. Of the 16 patients reported previously 12 had no evidence of disease at 31.7 months of mean follow-up. Although the number of patients was very small and the foHow-up period was short in all cases, local therapy including excision or external beam radiation with/without chemotherapy showed a good outcome in patients with local or locally invasive tumor. However, patients with disseminated disease showed a poor prognosis regardless of the kind of initial treatment. The cases of primary urethral lymphoma Treatment Followup Reference Radiation NED,9mos. [ 3) None Dead of unrelated cause [ 4) Excision, NED, IOyrs. [ 5) Chemotherapy Excision NED, 2 yrs . [ 6) Radiation, NED,9mos. [ 7) Chemotherapy Excision, Chemotherapy NED, 3 yrs. [ 8) Chemotherapy Unknown [ 9) Excision NED, 2 yrs. [to) TUR, NED, I yr. [to Radiation Chemotherapy NED,4yrs . [I 2) Radiation NED, 42 mos. [t3) Excision Dead, 5 mos . [t4) Palliative Dead, 2 mos. [t5) Chemotherapy NED,9mos. [t6) Chemotherapy Dead, 3 mos. [17) Chemotherapy NED, 6 mos. [t8) Radiation , Chemotherapy Dead, 7 mos . Presen tease 06) and 0 7) are males. NED=No evidence of disease SHIMIZU, et al.: Female urethral lymphoma 231 present patient died only seven months after diagnosis despite a good response of the primary tumor to external radiotherapy. According to the Rappaport grading system or the Working Formulation, diffuse histology and high grade tumor are considered to show a more unfavorable outcome than nodular subtype and intermediate grade tumors. In patients with these types of unfavorable histology or disseminated disease, early intensive chemo-therapy is recommended. REFERENCES 1) Suchi T, Tajima K, Nanba K, et al.: Some problems on the histopathological diagnosis of non-Hodgkin's malignant lymphoma: a proposal of a new type. Acta Pathol Jpn 29: 755-756, 1979 2) Freeman C, Berg JW and Cutler SJ: Occurrence and prognosis of external lymphomas. Cancer 29: 252-257, 1972 3) Grabstald H, Hilaris B, Henschke U, et al. : Cancer of the female urethra. JAMA 197: 835-842, 1966 4) Pak K, Takayama H, Tomoyoshi T, et al.: Reticulum cell sarcoma ofthefemale urethra. Acta Urol Jpn 26: 599-601, 1980 5) Nabholtz JM, Friedman S, Tremeaux JC, et al. : Non-Hodgkin's lymphoma of the female urethra: a rare extranodal entity. Gynecol Oncol 35: 110-111, 1989 6) Simpson RH, Bridger JE, Anthony PP, et al.: Malignant lymphoma of the lower urinary tract a clinicopathological study with review of the literature. Br J Urol 65: 254-260, 1990 7) Vapnek JM and Turzan CW: Primary malignant lymphoma of female urethra: report of a case and review of the literature. J Uro1147: 701-703, 1992 8) Kakizaki H, Nakada T, Sugano 0, et al.: Malignant lymphoma in the female urethra. Int J Urol 1: 281-282, 1994 9) Ohsawa M, Mishima K, Suzuki A, et al.: Malignant lymphoma of the urethra: report of a case with detection of Epstein-Bar virus genome in the tumor cells. Histopathology 24: 525-529, 1994 10) Khatib RA, Khalil AK, Tawil AN, et al.: Non-Hodgkin's lymphoma presenting as a urethral caruncle. Gynecol Oncol 50: 389-393, 1993 11) Melico MM, Latter R and Pierre-Louis C: Lymphoma of the female urethra masquerading as a caruncle. J Urol 108: 748-749, 1972 12) Touhami H, Brahimi S, Kubisz P, et al.: Non-Hodgkin's lymphoma of the female urethra. J Urol 137: 991-992, 1987 13) Selch MT, Mark RJ, Fu YS, et al.: Primary lymphoma of female urethra: long-term control by radiation therapy. Urology 42: 343-345, 1993 14) Watson EM, Sauer HR and Sadugor MG: Manifestations of the lymphoblastomas in the genito-urinary tract. J Urol 61: 626, 1949 15) Allen R and Nelson RP: malignancy: review of 22 cases. 547-550, 1978 Primary urethral South Med J 71 : 16) Chaitin BA, Manning JT and Ordonez NG: Hematologic neoplasms with initial manifestations in lower urinary tract. Urology 23: 35-42, 1984 17) Lopez AE, Latiff GA, Ciancio G, et al. : Lymphoma of urethra in patient with acquired immune deficiency syndrome. Urology 42: 596-598, 1993 18) Rajan N, Allman D, Scaglia B, et al.. Non-Hodgkin's lymphoma of the male urethra. J Urol 153: 1916--1917, 1995 (Received on August 16, 1996) Accepted on October 28, 1996 232 ActauroLJPn.voL43,No・3,1997和文抄録尿道原発 と考え られた悪性 リンパ腫の1例京都大学医学部泌尿器科学教室(主任:吉田 修教授)清水 洋祐,小 川 修,寺 地 敏郎岡田 裕作,吉 田 修82歳女性.主 訴は排尿困難と残尿感.膀 胱底部から尿道周囲にかけて鶏卵大の腫瘤を認め,生検にて非ポジキンリンパ腫と診断された.他臓器に病変を認めなかったため尿道原発の悪性リンパ腫 と診断し放射線療法を施行 した.治 療後,腫瘍は著明に縮小 したが肺転移が出現 した.エ トポシド単剤による化学療法を追加したが,放 射線治療後5カ 月で腫瘍の急激な増加を認め癌死 した.本 症例は国内外の文献上17例目の尿道原発悪性 リンパ腫 と考えられた.17例の治療成績をみると,限局性および局所浸潤例は切除,放 射線,化 学療法いずれも有効であったが,播 種性のものは治療法に関わらず予後不良であった.(泌尿紀要43:229-232,1997)

尿道原発と考えられた悪性リンパ腫の1例

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尿道原発と考えられた悪性リンパ腫の1例

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