The role of pelvic rehabilitation in quality of life in a patient harboring metastatic chordoma: a case report

Chordoma is a malignant tumor that usually involves the axial skeleton. They originate from remnants of the primitive notochord, with incidence of approximately 35% in clivus and 50% in sacrococcygeal region. Chordomas account for 1% of all primary brain tumors.4 The most affected group are middle-aged adults.5

Treatment is often difficult because the tumor is refractory to traditional cytotoxic agents and conventional radiotherapy, making block resection the main choice.6 However, the outcome of the treatment is significantly influenced by the size and location of the chordoma. Disease evolution may lead to severe quality of life impairment before death.7

We described previously an uncommon case of intratumoral bleeding in an intradural posterior fossa chordoma mimicking a spontaneous intraparenchymatous hemorrhage.1 Posteriorly, we described the diffuse spinal spread of tumor and subsequent symptoms. Now we discuss specifically the aims and results of pelvic rehabilitation of this patient.

This retrospective study which was submitted to Institutional Ethics and Human Research Committee approved under the CAEE (certificate do presentation of ethical appreciation) number 52340621.8.0000.0070 and under opinion No. 5.047.138.

Pelvic physical therapy procedure:

The physical evaluation was performed by the pelvic floor muscle assessment (PERFECT),15 graded from 0 to 5 was performed with movements such as: a) power, endurance, repetition and fast and every, contractions and timed reflexes of the region; b) muscle tone in the pelvic region with palpation; c) sacral sensitivity test and perineum region, d) lower limb motility test was also applied.16

In the evaluation, the patient presented: a) PERFECT with grade P0, E0, R0, F0, CS, TS the cocontraction was minimal; b) Perineal muscle tone was weak; c) Sensitivity test showed hyposensitivity in the region of the entire perineum (large and small vulvar lips), tingling paresthesia in the body of the perineum and pubic region and pain in the region close to the sacral region due to tumor damage; d) positive motor test without voluntary movement of the lower limbs.16

After the initial evaluation, urinary retention and severe constipation were detected (according to the Rome IV criteria).17

The patient was submitted to a rehabilitation program of the perineal region, every day, once a day and lasting approximately 45 minutes each session in her bed, totaling 6 face-to-face sessions plus guidance with the family member to be carried out at home. The sessions consisted of using electrotherapy (CARCI, São Paulo, Brazil) in FES mode (functional electrical stimulation) in the transverse region of the abdomen plus the perineal body region for learning perineal relaxation and were divided into two phases each of eight minutes with a second ON, one second OFF, and one second ON and two seconds OFF together guiding the importance of relaxation for urine output.18

Incomplete neural injuries may be common in oncologic pictures like the present case. In such cases, remaining neural stimuli become responsible for the innervation of a greater number of muscle fibers and produce less effective contractions, causing loss of urethral support.18

Electrical stimulation is believed to be able to increase intraurethral pressure through direct stimulation of the efferent nerves to the periurethral musculature, and by improving local blood flow.19 It is worth mentioning that the main objective of functional electrostimulation (FES) is to strengthen pelvic floor muscles, so it helps to improve urethral closing pressure by contracting more, if necessary. However, it does not prevent from urinary leakage. Thus, it is also necessary for the woman to acquire awareness of the perineal region. From the moment, she becomes aware of how to correctly contract the pelvic floor muscles; she will be able to produce an efficient contraction prior to any physical effort, which reduces the episodes of incontinence.19

The proposal made for this patient was divided into two stages: the first with rapid contractions in search of strengthening the pelvic floor and the other with longer relaxation time, prioritizing adequate relaxation for spontaneous urination and thus removing the use of the urinary catheter for relief.

In addition to the FES electrotherapy, electrotherapy was performed in TENS mode (transcutaneous electrical nerve stimulation) in the parasacral region to improve constipation. Before performing bladder catheterization, patient was encouraged to relax the region and try to urinate spontaneously. To do so, patient was kept seated and abdominal massages for fecal motility and bladder stimuli were also done.

The choice to use the parasacral ES was due to lower limbs motor deficits. Although many mechanisms are still uncertain regarding the use of electrostimulation (EE) or electroacupuncture, several studies suggest that it results in an increase in gastrointestinal motility, probably via reflex with activation of the supraspinal pathways that contribute to the effectiveness of intestinal function.20 21 It is known that the regulation of intestinal motility is influenced by the autonomic nervous system. While sympathetic predominantly inhibits the gastrointestinal tract and muscle activity, the parasympathetic system regulates propulsive colonic motility.18

In addition to the FES electrotherapy, electrotherapy was performed in TENS mode (transcutaneous electrical nerve stimulation) in the parasacral region to improve constipation.21 22 Always before relief bladder catheterization, the patient should relax the region and try to urinate in the diaper on her bed with the back of the trunk positioned at 90 degrees and abdominal massages for fecal motility and bladder stimuli at the time of spontaneous urination attempt.

The data with the functional improvement are shown in Table 1.

After the patient performed six pelvic physiotherapy sessions in order to bring quality of life to the urinary and fecal systems, she began to obtain positive results with an improvement in the urinary condition. After the patient left the hospital, she remained in remote contact with the physiotherapist for behavioral adjustments and daily exercises in the pelvic region, thus obtaining 100% improvement in her urinary condition. The fecal condition remained diagnosed with constipation.

The patient reported a great and expressive improvement in her quality of life as she returned to independence to perform her spontaneous bladder voiding.

Chordomas are rare, locally aggressive neoplasms of notochordal origin, accounting for 1% to 4% of all bone malignancies and 0.5% of all primary intracranial central nervous system.4 They characteristically tend to recur. In our case, a 33-year-old woman presented with acute new neurologic symptoms in a scenario of spinal cord metastatic intradural chordoma. With the advent of contemporaneous surgery, radiotherapy options and even available target therapy to treat chordomas (imatinib),22 patients may experience enlarged survival and thus face complications such as drop metastases along neuroaxis.1 2

The impact of rehabilitation and quality of life measures in such patients is almost unknown. Some papers have already pointed the challenges and impact of chordoma symptoms and treatment in quality of life, however up to date there is no report of specific rehabilitation program used in the course of disease.^[23,24]

Our case illustrates a late (7 years) follow-up presentation of an initial posterior fossa intradural chordoma. Probably due to surgical and adjuvant therapies, patient could experience a larger time of life and thus presented with bone and intradural tumoral spread, leading to motor and visceral symptoms. Even with limited survival time due to malignant nature of disease, it is possible to address specific symptoms such as pelvic ones and improve functions to reduce burden and improve quality of life.