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Keywords:

  • leiomyosarcoma;
  • renal pelvis;
  • rheumatoid pneumonia

Abstract

  1. Top of page
  2. Abstract
  3. Introduction
  4. Case report
  5. Discussion
  6. References

Abstract  A 54-year-old man presented at the Mibayashi clinic with bowel discomfort. Ultrasonography showed a left renal mass and the patient was referred to Noto General Hospital for urological evaluation. Results of the physical examination were unremarkable, but computed tomography and magnetic resonance imaging demonstrated a large tumor in the middle of the left kidney. The patient underwent left nephrectomy. On hemisection of the kidney, a firm tumor, measuring 8 × 7.5 cm, was seen occupying the renal pelvis. The histological diagnosis was leiomyosarcoma arising from the right renal pelvis. No treatment was provided after surgery and no recurrence was observed 6 months postoperatively.


Introduction

  1. Top of page
  2. Abstract
  3. Introduction
  4. Case report
  5. Discussion
  6. References

Leiomyosaroma of the renal pelvis is a rare disease. In Japan, with the exception of renal leiomyosarcoma arising from unknown lesions, only seven cases have been reported to date.1–6 The present paper discusses the diagnosis and treatment for leiomyosaroma of the renal pelvis.

Case report

  1. Top of page
  2. Abstract
  3. Introduction
  4. Case report
  5. Discussion
  6. References

A 54-year-old man presented at the Mibayashi clinic with bowel discomfort in April 2002. Ultrasonography showed a left renal mass. The patient was referred to Noto General Hospital for urological evaluation. Results of the physical examination were unremarkable. The serum erythrocyte sedimentation rate (ESR) was 52 mm/h (normal <10 mm/h), the lactate dehydrogenase (LDH) level was 449 IU/L (normal <220 IU/L) and the hemoglobin level was 13.2 g/dL (normal >13.5 g/dL). Drip infusion pyelography (DIP) showed compression of the left pyelogram. The patient complained of itching and coughing after DIP. Chest X-ray and computed tomography (CT) indicated rheumatoid pneumonia. Because of the iodide allergy, we could not use enhanced CT. Plain CT and magnetic resonance imaging demonstrated a large tumor in the middle of the left kidney (Fig. 1) without distant metastasis. Our clinical diagnosis was renal cell carcinoma. The patient underwent left nephrectomy. On hemisection of the kidney, the weight of the excised kidney was 532 g and a firm tumor, measuring 8 × 7.5 cm, was seen occupying the renal pelvis (Fig. 2). Histopathology disclosed homogeneously packed spindle tumor cells with cell atypia, pleomorphism and hyperchromatism (Figs 2,3). Immunohistochemical studies showed positive cystoplasmic staining of the tumor cells for smooth muscle-specific actin (Fig. 4). On the basis of these findings, a diagnosis of leiomyosarcoma of the renal pelvis was made. No treatment was provided following surgery. The patient was regularly followed up as an outpatient for more than 6 months without any evidence of recurrence by October 2002.

image

Figure 1. Plain computed tomography and T2-weighted magnetic resonance imaging revealed a large tumor in the middle of the right kidney.

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image

Figure 2. A firm tumor, measuring 8 × 7.5 cm, was seen occupying the renal pelvis.

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image

Figure 3. Histopathology disclosed homogeneously packed spindle tumor cells with cell atypia, pleomorphism and hyperchromatism (original magnification ×20).

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image

Figure 4. An immunohistochemical study revealed positive cystolasmic staining of the tumor cells for smooth muscle-specific actin (original magnification ×20).

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Discussion

  1. Top of page
  2. Abstract
  3. Introduction
  4. Case report
  5. Discussion
  6. References

Renal leiomyosarcoma may arise from the renal capsule or the smooth muscle fibers of the renal pelvis or vasculature.7 Leiomyosarcoma of the renal pelvis is rarer. To the best of our knowledge, only seven cases have been reported in Japan to date, except renal leiomyosarcoma arising from unknown lesions (Table 1).1–6 A differential diagnosis should include other malignant tumors and blood clotting in the renal pelvis. Preoperative diagnosis is very difficult, but one case has been reported for which fine needle aspiration cytology was found to be useful.8 As new techniques to aid in preoperative diagnosis, ureteroscopic inspection and biopsy may have been useful in making a preoperative diagnosis and deciding on a surgical method. Because no definitive treatment has been established for this rare disease, it is not known whether nephrectomy or nephreuretectomy is better. In Japan, nephrectomy has been used for five cases and nephreureterectomy for two (Table 1). Adjuvant chemotherapy has been used for three cases and radiation therapy has been used in one case (Table 1). Davis et al. recommend a wide surgical excision of the tumor followed by postoperative radiation and chemotherapy;9 however, Moudouni et al. reported 8 years survival after only wide surgical excision.10 Because of the rarity of this disease, we conclude that we cannot evaluate the relative usefulness of these different treatment modalities. The disease is, in fact, so rare that its prognosis is unknown. Not until many more cases have been documented can the prognosis become clear. To the best of our knowledge, ours is the first case of leiomyosarcoma of the renal pelvis accompanied by rheumatoid pneumonia. The relationship between leiomyosarcoma of the renal pelvis and rheumatoid pneumonia remains unknown.

Table 1.  Seven cases of leiomyosarcoma reported in Japan
ReferenceAge (years)SexSymptomsSideKidney weight (g)Treatment
239MaleGross hematuria and right flank painRight490Nephrectomy and post-radiation
319FemaleLeft flank painLeft650Nephrectomy and adjuvant chemotherapy
465MaleGross hematuriaLeft284Nephrectomy
667MaleGross hematuria and left flank painLeft150Nephreureterectomy and adjuvant chemotherapy
565MaleLeft flank painLeft980Nephrectomy and adjuvant chemotherapy
759FemaleGross hematuriaRight150Nephreureterectomy
Present case54MaleBowel discomfortLeft532Nephrectomy

References

  1. Top of page
  2. Abstract
  3. Introduction
  4. Case report
  5. Discussion
  6. References
  • 1
    Minami T, Oishi Y, Sasaki T, Saito K, Minami T. The case of the renal leiomysarcoma. Jpn. J. Urol. 1970; 61: 515.
  • 2
    Chen Z, Machida T, Masuda F et al. Leiomyosarcoma of the kidney: Report of two cases. Jpn. J. Urol. 1978; 69: 1512.
  • 3
    Kanamaru H, Sasaki M. A case of renal leiomyosarcoma. Acta Urol. Jpn. 1983; 29: 15214.
  • 4
    Imamura A, Kakinoki T. Renal leiomyosarcoma: A case report. Nihinihon J. Urol. 1988; 50: 6958.
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    Nakachi K, Doi Y, Kuroda J. Renal leiomyosarcoma: A case report. Nihinihon J. Urol. 1989; 51: 12257.
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    Tohda A, Nagano T, Yamaguchi S, Koide T, Ohnishi S. Leiomyosarcoma of the renal pelvis: A case report. Nihinihon J. Urol. 1992; 54: 111821.
  • 7
    Nair J, Thomas JA. Leiomyosarcoma of the kidney: A characterization. Indian J. Cancer 1977; 14: 284.
  • 8
    Chow LT-C, Chan S-K, Chow W-H. Fine needle aspiraton cytodiagnosis of leiomyosarcoma of the renal pelvis. Acta Cytol. 1994; 38: 75963.
  • 9
    Davis R, Vaccaro JA, Hodes GF, Belville WD, Kieling V. Renal leiomyosrcoma: Plea aggressive therapy. Urology 1992; 40: 16871.
  • 10
    Moudouni SM, En-Nia I, Rioux-Leclerq N, Guille F, Lobel B. Leiomyosarcoma of the renal pelvis. Scand. J. Urol. Nephrol. 2001; 35: 4257.