Skip to main content

Advertisement

We’d like to understand how you use our websites in order to improve them. Register your interest.

Pleomorphic rhabdomyosarcoma of the liver in an adult: a rare case report

Abstract

Background

Rhabdomyosarcoma (RMS), a malignant neoplasm that normally differentiates to form striated muscle, is the most common type of childhood soft tissue sarcoma. However, it infrequently occurs in adults and is uncommon in the liver. We herein report a case of RMS of the liver in an adult.

Case presentation

A 73-year-old woman was admitted to our institution for investigation of a hepatic mass. She had been followed for primary biliary cirrhosis for the past 20 years. A contrast-enhanced computed tomography scan of the abdomen showed a 12- × 10-cm heterogeneous low-density mass lesion containing cystic and solid components. A percutaneous liver biopsy was performed, and poorly differentiated cancer containing an RMS cell-like component was observed. The patient was diagnosed with RMS of the liver, and open surgery with right hepatic lobectomy was performed. Histopathological examination confirmed a diagnosis of pleomorphic RMS of the liver. The patient died of rapid progression of the tumor 6 months after the operation.

Conclusions

The tumor site in the present case is rare. The details of this case add to the current evidence base regarding establishment of the standard diagnosis and treatment of this rare condition. We recommend consideration of RMS as a differential diagnosis for hepatic tumors.

Peer Review reports

Background

Rhabdomyosarcoma (RMS) is a malignant neoplasm that normally differentiates to form striated muscle. RMS is the most common type of childhood soft tissue sarcoma, constituting 5 to 10% of all solid tumors in childhood. However, it rarely occurs in adults; soft tissue sarcomas account for less than 1% of all cancers in adults [1,2,3]. Although this tumor may occur anywhere in the body, it is uncommon in the liver.

We herein report the clinicopathological features of a case of RMS of the liver in a 73-year-old woman.

Case presentation

A 73-year-old woman presented with a fever and a 2-month history of right upper abdominal pain. The patient had been followed for primary biliary cirrhosis for the past 20 years and was being treated with ursodeoxycholic acid. A computed tomography (CT) scan performed by the previous doctor revealed a liver abscess, which was drained from the right hypochondriac region; however, the patient’s symptoms did not improve. She was admitted to our institution for further investigation of a hepatic mass. Physical examination revealed a right upper abdominal mass, but no anemia or jaundice.

Laboratory data showed an elevated C-reactive protein level (7.6 mg/dL). The hemoglobin concentration, white blood cell count, platelet count, electrolyte levels, liver enzyme levels, and bilirubin level were within the reference range. The serum levels of α-fetoprotein and PIVKA-II were 4 ng/mL and 43 U/mL, respectively.

An abdominal contrast-enhanced CT scan revealed a 12- × 10-cm heterogeneous low-density mass lesion containing cystic and solid components with post-contrast enhancement in the solid component (Fig. 1a, b). This mass occupied the right lobe of the liver, and a large component of the lesion was present in the right subhepatic space. We determined that the tumor originated in the liver because a CT scan performed for follow-up of the patient’s primary biliary cirrhosis 4 months previously had revealed a 2-cm low-density tumor in liver segment 6 (Fig. 1c). A percutaneous liver biopsy was performed, and poorly differentiated cancer containing an RMS cell-like component was diagnosed.

Fig. 1
figure1

Abdominal contrast-enhanced computed tomography showing a heterogeneous mass lesion in the right lobe of the liver; a axial section, b coronal section. c Four months previously, a 2-cm low-density tumor (arrow) was detected in liver segment 6

The patient underwent open surgery with right hepatic lobectomy. The intraoperative findings confirmed a tumor occupying the right lobe of the liver and no infiltration of the surrounding organs (Fig. 2). Examination of the gross specimen revealed a multilobulated tumor with a solid component (Fig. 3a, b). Histopathological examination of the tissue showed haphazardly oriented, large and small irregularities and pleomorphic or round cells containing abundant and eccentric eosinophilic cytoplasm and small oval nuclei with a prominent nucleolus. Immunohistochemical analysis showed desmin, myogenin, and myoglobin positivity and cytokeratin negativity (Fig. 4a–d). Based on these findings, the patient was diagnosed with pleomorphic RMS of the liver.

Fig. 2
figure2

Intraoperative view of resected mass

Fig. 3
figure3

(a) Postoperative specimen of rhabdomyosarcoma of the liver. b Cut surface showed a tan-brown solid, friable tumor

Fig. 4
figure4

(a, b) Histopathological examination showed pleomorphic nuclei and spindle cells. Immunohistochemical analysis revealed (c) desmin positivity and (d) myogenin positivity

No complications occurred in the postoperative period, and the patient was discharged on the 28th postoperative day. Two months after the operation, an abdominal CT scan showed an 8-cm low-density tumor in the liver resection area compressing the inferior vena cava and peritoneal dissemination in the drainage route for diagnosis of the liver abscess before admission to our institution (Fig. 5a, b). The patient received one course of 70% dose trabectedin. Despite an initial good response to chemotherapy, she complained of severe adverse effects including loss of appetite and fatigue, and she rejected further chemotherapy. She subsequently experienced rapid progression of the tumor and died of malnutrition and multiple organ failure 6 months after the operation.

Fig. 5
figure5

Computed tomography showed 8-cm low-density tumor compressing the inferior vena cava in the (a) liver resection area and (b) peritoneal space (arrow)

Discussion and conclusions

RMS in the liver, especially that in adults, is difficult to manage because of the absence of standard diagnostic criteria or a standard treatment protocol. Only 10 cases of RMS of the liver in adults, including our case, have been reported to date and are summarized in Table 1 [4,5,6,7,8,9,10,11,12]. Among these cases, RMS was more common in men than women, and our case involved the oldest patient.

Table 1 Reported cases of rhabdomyosarcoma of the liver in adults

Horn and Enterline et al. [13] reported four subgroups of RMS: embryonal, alveolar, pleomorphic, and botryoid. Botryoid RMS is actually a subtype of embryonal RMS [14]. Embryonal RMS is the most frequent type of RMS in young children, alveolar RMS is the most frequent type in patients older than 10 years, and pleomorphic RMS is the most frequent type in advanced-age adults [3, 13]. Among the adult patients in whom RMS originated in the liver, four had embryonal RMS and three had pleomorphic RMS.

No reports to date have described the typical imaging findings and symptoms of RMS. Most reported cases were detected as a large mass of > 10 cm in diameter occupying a liver lobe. Our patient had a 12-cm liver mass, initially diagnosed and treated as a liver abscess, that caused peritoneal dissemination in line with the drainage route after resection. In the investigation of such cases, it is important to perform a percutaneous biopsy and include RMS as a differential diagnosis for liver masses in adults.

RMS in adults is a highly malignant tumor with a poor prognosis because of the absence of a standard treatment protocol. Sultan et al. [15] reported that RMS in adults had significantly poorer outcomes than in childhood (mean 5-year overall survival rates, 27% ± 1.4 and 61% ± 1.4%, respectively; P < 0.0001). Among previously reported cases of RMS originating in the liver, only two patients survived longer than 12 months; most patients died within 12 months from onset of the initial symptoms. It is necessary to establish the optimal treatment protocol and thus improve the outcome of patients with this rare but fatal cancer.

Radical resection with negative margins, chemotherapy, and radiotherapy are suggested by the Intergroup Rhabdomyosarcoma Study Group; these interventions constitute the generally optimal treatment protocol in childhood [16, 17]. Chemotherapeutic drugs include actinomycin D, vincristine, doxorubicin, cyclophosphamide, etoposide, and ifosfamide. We treated our patient’s RMS with trabectedin, as for other soft tissue sarcomas, because multi-drug combination therapy is considered difficult because of the worsening performance status. Our patient initially showed a good response to chemotherapy; however, she could not continue further chemotherapy because of severe adverse effects.

We have herein reported an extremely rare case of pleomorphic RMS of the liver in an adult. The rarity of this case is due to the location of the tumor and the age of the patient, and its reporting will help to establish standard diagnosis and treatment.

Availability of data and materials

The datasets used and/or analyzed during the current study are available from the corresponding author on reasonable request.

Abbreviations

RMS:

Rhabdomyosarcoma

CT:

Computed tomography

References

  1. 1.

    Goldblum JR, Weiss SW, Folpe AL. Enzinger and Weiss’s soft tissue tumors e-book. Philadelphia: Elsevier Health Sciences; 2013.

    Google Scholar 

  2. 2.

    Ulutin C, Bakkal H, Kuzhan O. A cohort study of adult rhabdomyosarcoma: a single institution experience. World J Med Sci. 2008;3:54–9.

    Google Scholar 

  3. 3.

    Tutar NU, Cevik B, Otgun I, Tarhan NC, Ozen O, Coskun M. Primary embryonal botryoid-type rhabdomyosarcoma of the liver. Eur J Radiol Extra. 2007;61:5–7.

    Article  Google Scholar 

  4. 4.

    Miller TR, Pack GT. Total right hepatic lobectomy for rhabdomyosarcoma. AMA Arch Surg. 1956;73:1060–2.

    Article  CAS  Google Scholar 

  5. 5.

    Goldman RI, Freiedman NB. Rhabdomyosarcohepatoma in an adult and embryonal hepatoma in a child. Am J Clin Pathol. 1969;51:137–43.

    Article  CAS  Google Scholar 

  6. 6.

    Watanabe A, Mori M, Mizobuchi K, Hara I, Nishimura K, Nagashima H. An adult case with rhabdomyosarcoma of the liver. Jpn J Med. 1983;22:240–4.

    Article  CAS  Google Scholar 

  7. 7.

    McArdle JP, Hawley I, Shevland J, Brain T. Primary embryonal rhabdomyosarcoma of the liver. Am J Surg Pathol. 1989;13:961–5.

    Article  CAS  Google Scholar 

  8. 8.

    Akasofu M, Kawahara E, Kaji K, Nakanishi I. Sarcomatoid hepatocellular-carcinoma showing rhabdomyoblastic differentiation in the adult cirrhotic liver. Virchows Arch. 1999;434:511–5.

    Article  CAS  Google Scholar 

  9. 9.

    Schoofs G, Braeye L, Vanheste R, Verswijvel G, Debiec-Rychter M, Sciot R. Hepatic rhabdomyosarcoma in an adult: a rare primary malignant liver tumor. Case report and literature review. Acta Gastroenterol Belg. 2011;74:576–81.

    PubMed  CAS  Google Scholar 

  10. 10.

    Aassab R, Kharmoume S, Mahfoud T, Khmamouche MR, M’rabti H, Errihani H. Primary embryonal botryoid-type rhabdomyosarcoma of the liver in adult: case report and review of the literature. Afr J Cancer. 2012;2:124–6.

    Article  Google Scholar 

  11. 11.

    Arora A, Jaiswal R, Anand N, Husain N. Primary embryonal rhabdomyosarcoma of the liver. BMJ Case Rep. 2016;2016:bcr2016218292.

  12. 12.

    Yin J, Liu Z, Yang K. Pleomorphic rhabdomyosarcoma of the liver with a hepatic cyst in an adult: case report and literature review. Medicine. 2018;97:e11335.

    Article  Google Scholar 

  13. 13.

    Horn RC, Enterline HT. Rhabdomyosarcoma: a clinicopathologic study and classification of 39 cases. Cancer. 1958;11:181–99.

    Article  Google Scholar 

  14. 14.

    Nakhleh RE, Swanson PE, Dehner LP. Juvenile (embryonal and alveolar) rhabdomyosarcoma of the head and neck in adults: a clinical, pathologic, and immunohistochemical study of 12 cases. Cancer. 1991;67:1019–24.

    Article  CAS  Google Scholar 

  15. 15.

    Joshi D, Anderson JR, Paidas C, Breneman J, Parham DM. Crist W; soft tissue sarcoma Committee of the Children’s oncology group. Age is an independent prognostic factor in rhabdomyosarcoma: a report from the soft tissue sarcoma Committee of the Children’s oncology group. Pediatr Blood Cancer. 2004;42:64–73.

    Article  CAS  Google Scholar 

  16. 16.

    Sultan I, Qaddoumi I, Yaser S, Rodriguez-Galindo C, Ferrari A. Comparing adult and pediatric rhabdomyosarcoma in the surveillance, epidemiology and end results program, 1973 to 2005: an analysis of 2,600 patients. J Clin Oncol. 2009;27:3391–7.

    Article  Google Scholar 

  17. 17.

    Pizzo PA, Poplack DG. Principles and practice of pediatric oncology. Philadelphia: Lippincott Williams & Wilkins; 2015.

    Google Scholar 

Download references

Acknowledgments

We are grateful to the members of the Department of Gastroenterologic Surgery of Kanazawa University for their helpful suggestions. We also thank Angela Morben, DVM, ELS, from Edanz Group (https://en-author-services.edanzgroup.com/), for editing a draft of this manuscript.

Funding

The authors declare that they received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.

Author information

Affiliations

Authors

Contributions

MO and HT assembled, analyzed, and interpreted the patient’s data and case presentation. YO, HS, SN, and IM reviewed the literature. HT, IN, SF, KO, and TO edited and critically revised the manuscript for intellectual content. All authors contributed to the writing of the manuscript. All authors read and approved the final manuscript.

Corresponding author

Correspondence to Mitsuyoshi Okazaki.

Ethics declarations

Ethics approval and consent to participate

Not applicable.

Consent for publication

Written informed consent was obtained from the patient’s husband for publication of this case report and any accompanying images.

Competing interests

The authors declare that they have no competing interests.

Additional information

Publisher’s Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.

Reprints and Permissions

About this article

Verify currency and authenticity via CrossMark

Cite this article

Okazaki, M., Tajima, H., Ohbatake, Y. et al. Pleomorphic rhabdomyosarcoma of the liver in an adult: a rare case report. BMC Surg 20, 81 (2020). https://doi.org/10.1186/s12893-020-00742-7

Download citation

Keywords

  • Pleomorphic rhabdomyosarcoma
  • Heterogeneous mass in liver
  • Right hepatic lobectomy
  • Case report