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Urol Case Rep. 2019 Sep; 26: 100958. Published online 2019 Jul 2. doi: 10.1016/j.eucr.2019.100958

PMCID: PMC6614174PMID: 31321211

Advanced bladder cancer with malignant psoas syndrome: A case report with a focus on physical
findings and complications

Katsuki Tsuchiyama,a,∗ Hideaki Ito,a Masaya Seki,a Kunihiro Inai,b and Osamu Yokoyamaa

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Associated Data

Supplementary Materials

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Abstract

Malignant psoas syndrome (MPS) is a rare clinical condition caused by cancer invasion of the iliopsoas
muscle and has very poor prognosis. We report a case involving a 58-year-old woman with bilateral MPS
caused by advanced bladder cancer. Rapid progress of a severe crouching posture with multiple deep
venous thromboses was an important symptom of this case. Although 4 cycles of chemotherapy were
administered, the patient died 8 months following disease onset. Since, these noteworthy symptoms
have never been previously reported, in this report, we present the characteristic physical findings using
photographs and cancer-related events that occur in MPS.

Keywords: Malignant psoas syndrome, Bladder cancer

Abbreviations: MPS, malignant psoas syndrome

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Introduction

Malignant psoas syndrome (MPS) is a rare cancer-associated complication caused by tumor infiltration
to the iliopsoas muscle.1 Painful fixed flexion of the hip joint and lumbosacral plexopathy are important
signs of MPS. Many of the MPS-related case reports mention the importance of palliative treatment for
refractory cancer pain.1, 2, 3 It has also been pointed out that the diagnosis of MPS may be delayed
because many medical personnel are unaware of the syndrome and its characteristic symptoms.2 We
report a case of bilateral MPS involving severe symptoms, with a focus on characteristic physical findings
and cancer-related events caused by MPS.

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Case presentation

A 58-year-old woman visited the orthopedic department of our hospital presenting with a one month-
history of a gait disorder with right leg pain. Intense pain and fixed flexion of the right hip joint was
noted. She was unable to stand and walk independently at the first visit. Computed tomography (CT)
revealed a muscle-invasive bladder tumor (Fig. 1A) and extensive metastatic lesions invading the
iliopsoas muscle (Fig. 1B); she was then referred to our department.

Fig. 1

Fig. 1

(A, B, C) Computed tomography at the first visit showing a thrombus in the inferior vena cava (red
arrow); (D) Computed tomography after 4 cycles of chemotherapy. (For interpretation of the references
to color in this figure legend, the reader is referred to the Web version of this article.)

Cystoscopy showed large, solid, non-papillary tumors from the posterior wall to the right-sided wall of
the bladder. Transurethral resection was performed for pathological diagnosis which revealed high-
grade urothelial carcinoma. Systemic chemotherapy with gemcitabine and carboplatin was
administered, due to her renal hypofunction caused by hydronephrosis. In addition, extensive deep
venous thromboses (DVT) were observed below the inferior vena cava (Fig. 1C); therefore,
anticoagulation therapy using warfarin was initiated.
After 2 cycles of chemotherapy, the tumor size (diameter) reduced by 10%. However, after 2 additional
cycles of chemotherapy, a marked increase in tumor size was observed (Fig. 1D); an increase in DVT was
also noted. The contracture of the bilateral hip joint was exacerbated; therefore, the patient was unable
to sit on the bed without assistance due to discomfort (Fig. 2A). Chemotherapy was discontinued due to
cancer progression. She died 2 months after chemotherapy termination (8 months post-onset).

Fig. 2

Fig. 2

(A) Photograph of the patient at the end of chemotherapy. (B) Macroscopic view of excessive flexion of
lower extremities just before postmortem investigation.

We performed an autopsy to investigate the reasons for rapid cancer progression and flexion
contractures of the hip joints (Fig. 2B). Fig. 3A shows the spread of a disseminated tumor in the
retroperitoneal cavity. Macroscopically, the tumor consisted of numerous lymph nodes sized ≤1 cm;
these lymph nodes fused with and replaced the psoas major muscle. Histopathologically, the tumor was
a poorly differentiated urothelial carcinoma showing differentiation into squamous cell carcinoma and
adenocarcinoma with signet ring cells (Fig. 3B and C).

Fig. 3

Fig. 3

(A) Horizontal section at the level of the kidneys showing multiple extraperitoneal lymph nodes (red
arrowheads). (B) Microscopic examination of a hematoxylin–eosin-stained specimen showing an area of
differentiation into squamous cell carcinoma and (C) an area of differentiation into adenocarcinoma. Ao:
aorta; IVC: inferior vena cava.. (For interpretation of the references to color in this figure legend, the
reader is referred to the Web version of this article.)

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Discussion

The major characteristics of MPS are i) lumbosacral plexopathy, ii) painful hip flexion, and iii) malignant
involvement of the psoas major muscle. Since the first report of an MPS case by Stevens et al., in 1990,
40 cases, including ours, have been reported in the English literature.1 MPS has a very poor prognosis
because it occurs in the advanced stages of cancer; many cases have reported a prognosis for survival of
0.5–36 months after MPS diagnosis. Refractory pain due to cancer invasion is often involved, and many
reports have mentioned the importance of palliative treatment for cancer pain. However, few case
reports have described the characteristic physical findings or psoas muscle invasion-related symptoms
observed in patients with MPS.

In this report, Fig. 2A and B provide visual references demonstrated the characteristic physical posture
observed in patients with MPS. Because the patient's bilateral hip joints were strongly flexed, she had to
spend time in a crouching position. The autopsy revealed that metastatic lymph node cancer had spread
not only to the iliopsoas muscle but also to the entire pelvis, with remnants of little muscle fibers. Such
extensive spread of the disease was considered to be a reason for severe flexion and contraction of the
bilateral hip joint.

In this case, the tumor put pressure on the inferior vena cava, causing an extensive DVT on the caudal
side of the tumor. The risk of coagulopathy has been reported to increase in patients with advanced
cancers. Patients may develop cerebral embolization, venous thrombosis or other symptoms resulting
from cancer-related hypercoagulation, referred to as Trousseau's syndrome.4 In addition to cancer-
associated thrombosis, direct compression of the inferior vena cava by the tumor in the iliopsoas muscle
and long-term underlying conditions may increase thrombogenic risks in patients with MPS. Mizoguchi
et al., reported that DVTs associated with colorectal cancer improved with use of anticoagulant therapy
and tumor resection.5 However, providing treatments other than anticoagulation therapy to patients
with MPS is difficult because of progressive disease. In this case, no improvement was observed in
patients with DVT even after administering anticoagulation therapy. Therefore, the patient developed
severe edema in both lower limbs, resulting in a further decrease in activities of daily living.

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Conclusion

We presented the typical physiological findings and complications related to MPS. We suggest that
attention be paid to not only cancer pain but also to other complications, such as DVTs, while treating
patients with MPS. Furthermore, our findings show that these cancer-related events can occur in a short
period.

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Conflicts of interest

None.
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Consent

Written informed consent was obtained from the patient's family.

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Funding

This research did not receive any specific grant from funding agencies in the public, commercial, or not-
for-profit sectors.

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Acknowledgements

None.

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Footnotes

Appendix ASupplementary data to this article can be found online at

https://doi.org/10.1016/j.eucr.2019.100958.

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Appendix A. Supplementary data

The following is the Supplementary data to this article:

Multimedia component 1:

Click here to view.(271 bytes, xml)Multimedia component 1

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References

1. Stevens M.J., Gonet Y.M. Malignant psoas syndrome: recognition of an oncologic entity. Australas
Radiol. 1990;34:150–154. [PubMed] [Google Scholar]

2. Takamatsu S., Murakami K., Takaya H. Malignant psoas syndrome associated with gynecological
malignancy: three case reports and a review of the literature. Mol Clin Oncol. 2018;9:82–86. [PMC free
article] [PubMed] [Google Scholar]

3. Ampil F.L., Lall C., Datta R. Palliative management of metastatic tumors involving the psoas muscle:
case reports and review of the literature. Am J Clin Oncol. 2001;24:313–314. [PubMed] [Google Scholar]

4. Trousseau A. Plegmasia alba dolens. Clinique Medicale de l'Hotel-Dieu Paris. 1865;3:654–712. [Google
Scholar]

5. Mizoguchi S., Sawai T., Hirota A. Trousseau's syndrome causing refractory deep venous thrombosis.
Intern Med. 2018;57:623–626. [PMC free article] [PubMed] [Google Scholar]

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NIH NLM LogoLog in

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endorsement of, or agreement with, the contents by NLM or the National Institutes of Health. Learn
more about our disclaimer.

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N Am J Med Sci. 2010 Jun; 2(6): 285–287.

PMCID: PMC3347637PMID: 22574305

A large retroperitoneal tumor with psoas infiltration: A rare case report

Subhash Goyal, MS, MAMS, FAIS, FICS,1 Mahesh Gupta, MS, DMAS, FMAS,1 Rikki Singal, MS, FICS,1
Rekha Goyal, MD,2 and Amit Mittal, MD2

Author information Copyright and License information Disclaimer

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Abstract

Context:

Retroperitoneal sarcomas are rare neoplasms. Computed Tomography or Magnetic Resonance Imaging
is performed in patients with these tumors to detect local extent and distant metastases of the tumor
and for preoperative surgical planning. However the evaluation and treatment of retroperitoneal
sarcomas are challenging because of its rarity and frequent presentation with advanced disease in an
anatomically complex location.

Case Report:

We report a case of large retroperitoneal tumor in a female on the right flank. Ultrasound and
Computed tomography scan of the abdomen revealed infiltration into the psoas muscle on the same
side along with displacement of adjacent structures. Ultrasound guided fine needle aspiration cytology
was suggested of liposarcoma. Surgical resection of the tumor was done. In follow up six months,
patient was asymptomatic.

Conclusions:

In case of retroperitoneal tumors CT scan remains the diagnostic modality of choice and the inefficiency
of adjuvant therapy; high recurrence rate and the very low chance of curing the patient after recurrence
make the prognosis of these patients poor. However surgical resection remains the optimum treatment
in all cases of retroperitoneal tumors.

Keywords: Sarcoma, malignancy, fibrous histiocytoma, surgery

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Introduction

Retroperitoneal tumor constitutes only 10%-20% of all the sarcomas with an incidence of 0.3%-0.4% per
100000 population[1]. Although they may affect any age group, the peak incidence is found in 5th
decade. These malignant tumors arise from mesenchymal cells and are usually located in muscle, fat,
and connective tissues. They have varying clinical courses depending on their histological subtype and
grade and are usually very large at the time of presentation[2]. CT and MRI are useful investigations to
assess its extent and preoperative planning. The treatment options include radiotherapy, chemotherapy
and surgical resection, however surgery is found to be the optimum treatment.

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Case Report
A 48-year old woman presented with a painful swelling in right flank of 5 month duration. Local
examination revealed a non-tender, lobulated, firm mass of about 18 × 15 cm in size in the right flank.
Routine investigations were normal.

Abdominal ultrasonography (USG) revealed a primary retroperitoneal mass with liver and right kidney
were pushed anterio-superiorly and pancreas anteriorly.

Computed tomography (CT) of the abdomen showed a huge heterogeneous mass lesion of size 20cm ×
13cm × 11cm in the retroperitoneal region on the right side extending superiorly up to the inferior
surface of right lobe of liver, inferiorly up to the level of upper border of S1 vertebra. Laterally it
extended just before the right lateral abdominal wall while medially it was abetting the aorta. Right
kidney was displaced anterior-superiorly while the bowel loops and inferior vena cava were pushed
anteromedially. The right psoas muscle was not separately visualized and was suggestive of infiltration.
The fat planes between the mass and muscles of posterior abdominal wall and at places between the
inferior surfaces of liver were also not well visualized suggestive of infiltration.

Post contrast study revealed strong heterogeneous enhancement with non enhancing areas due to
necrosis. No evidence of calcification was seen in the mass. The left kidney, pancreas and spleen and
aorta appeared normal. Gall bladder was contracted without any lymphadenopathy. There was no bone
erosion. Renal vein and artery were normal.

USG guided fine needle aspiration was done which showed liposarcoma.

On exploration there was a large encapsulated lobulated, firm tumor occupying the right half of the
abdominal cavity which was adherent to the right kidney and infiltrating into the psoas major muscle.
Tumor was separated from the aorta and kidney after adhenolysis. Debulking of the tumor was done
and the resected specimen measured about 20 × 12 cm in size (Figs. (Figs.11–3).

An external file that holds a picture, illustration, etc.

Object name is NAJMS-2-285-g001.jpg

Fig. 1
Gross specimen showing anterior view of retroperitoneal sarcoma. The right kidney was displace
anterosuperiorly by the mass and the bowel loops and inferior vena cava were pushed anteromedially.
The right psoas muscle was not separately visualized due to infiltration.

An external file that holds a picture, illustration, etc.

Object name is NAJMS-2-285-g003.jpg

Fig. 3

The cut section of specimen showing features of malignant sarcoma in the retroperitoneum.

An external file that holds a picture, illustration, etc.

Object name is NAJMS-2-285-g002.jpg

Fig. 2

Gross specimen showing posterior view of retroperitoneal sarcoma extending superiorly up to the
inferior surface of the liver and inferiorly up to the S1 vertebra. Laterally it extended just before the right
lateral abdominal wall while medially it was abetting the aorta.

Histopathological findings revealed presence of numerous tumor giant cells along with numerous
inflammatory cells consisting of polymorphs, lymphocytes and plasma cells and the diagnosis of
inflammatory malignant fibrous histiocytoma was made. Patient is asymptomatic on subsequent follow-
up of 6 months.

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Discussion

Retroperitoneal sarcomas are rare tumors accounting for only 1%–2% of all solid malignancies arising
from mesenchymal cells, which are usually located in muscle, fat, and connective tissues[2,3]. In
different studies the most common types of retroperitoneal soft tissue sarcomas in adults vary and the
most commonly encountered histologic subtypes of retroperitoneal sarcoma remain liposarcoma(41%),
leiomyosarcoma (28%), malignant fibrous histiocytoma (7%), fibrosarcoma (6%), and malignant
peripheral nerve sheath tumor (3%)[3].
Commonest clinical presentation is an abdominal mass because of the fact that retroperitoneal sarcoma
smaller than 5 cm usually remain silent. The only signs and symptom if any are non-specific e.g. vague
abdominal discomfort or pain, weight loss, and early satiety where as some cases may present with
intestinal obstruction or bleeding. Lower extremity swelling or pain has also been reported as presenting
symptoms[4].

CT remains the diagnostic modality of choice in suspected retroperitoneal sarcoma. It facilitates

accurate estimation of tumor location and size, its relationship to surrounding anatomic structures, and
identification of metastatic lesions within the abdomen[5].

Primary radiation therapy for cure is seldom effective for retroperitoneal sarcomas but can provide
palliation in selected cases. Systemic chemotherapy for chemo sensitive lesions, such as poorly
differentiated liposarcoma, malignant fibrous histiocytoma (MFH), synovial cell sarcoma, and primitive
neuroectodermal tumors (PNET), can be useful when used in a neoadjuvant manner[6]. Consequently,
surgical resection is the mainstay of treatment for retroperitoneal sarcomas and requires enbloc
resection of the primary tumor[7].

In case the tumor cannot be separated from the adjacent organs then the resection includes tumor
along with the organ involved such as colon, small bowel, kidney, adrenal, and pancreas[8].
Postoperative adjuvant chemotherapy and radiation have not been proven to be of any additional
benefit. Overall prognosis is influenced by tumor size, grading and resected margins. CT scans at every 6-
month intervals can be of immense value to rule out any recurrence because these tumors usually recur
within 2 years of initial surgical resection[9].

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Conclusion

A rare case of retroperitoneal tumor of huge size infiltrating the psoas muscle is reported. Inflammatory
malignant fibrous histiocytoma is extremely rare. However all the retroperitoneal tumors should be
subjected to surgical excision to achieve optimum results.

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References
1. Mettlin C, Priore R, Rao U. Results of the national soft tissue sarcoma registry. J Surg Oncol.
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2. Francis IR, Cohan RH, Varma DGK, Sondak VK. Retroperitoneal sarcomas. Cancer Imaging.
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5. Neville A, Herts BR. CT Characteristics of Primary Retroperitoneal Neoplasms. J Cardiovasc Mag Reson.
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7. Gholami S, Jacobs CD, Kapp DS, Parast LM, Norton JA. The Value of Surgery for Retroperitoneal
Sarcoma. Sarcoma. 2009;2009:605840. [PMC free article] [PubMed] [Google Scholar]

8. Xu YH, Guo KJ, Guo RX, Ge CL, Tian YL, He SG. Surgical management of 143 patients with adult primary
retroperitoneal tumor. World J Gastroenterol. 2007 May 14;13(18):2619–2621. [PMC free article]
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9. Gupta AK, Cohan RH, Francis IR, Sondak VK, Korobkin M. CT of Recurrent Retroperitoneal Sarcomas.
AJR. 2000;174:1025–1030. [PubMed] [Google Scholar]

Articles from North American Journal of Medical Sciences are provided here courtesy of Wolters Kluwer
-- Medknow Publications

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