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| CLINICAL ARTICLE |
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| Year : 2021 | Volume
: 22
| Issue : 2 | Page : 131-136 |
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Care of patient with desmoid tumour
Ida Nirmal1, Latha Madan2
1 Professor, College of Nursing, CMC, Vellore, Tamil Nadu, India
2 M.Sc (N) Student, College of Nursing, CMC, Vellore, Tamil Nadu, India
| Date of Submission | 23-May-2020 |
| Date of Decision | 05-Mar-2021 |
| Date of Acceptance | 18-Sep-2021 |
| Date of Web Publication | 31-Jan-2022 |
Correspondence Address:
Mrs. Ida Nirmal
College of Nursing, CMC, Vellore, Tamil Nadu
India
 Source of Support: None, Conflict of Interest: None
DOI: 10.4103/IJCN.IJCN_39_20
Desmoid tumours are uncommon mesenchymal neoplasms with a fibrotic band-like consistency. They are also called aggressive fibromatosis tumours which are locally aggressive with no potential for distant metastases. The important causes of morbidity and mortality are local recurrence and adjacent organ involvement. Desmoids can be of three types, namely intra-abdominal, in the abdominal wall, or extra-abdominal. Treatment options include observation, surgical resection, radiotherapy, conventional chemotherapy, hormonal agents and newer molecular-targeted agents. A multidisciplinary approach tailored to the individual patient is usually needed, depending on the location, local effects and the clinical course of the disease.
Keywords: Aggressive fibromatosis, desmoid tumour, familial adenomatous polyp, musculoaponeurotic fibromatosis, non-metastasizing fibrosarcoma
How to cite this article:
Nirmal I, Madan L. Care of patient with desmoid tumour. Indian J Cont Nsg Edn 2021;22:131-6 |
| Introduction | |  |
The term desmoid originates from 'desmos' meaning tendon-like. This term was coined by Muller in 1838. Desmoid tumours are benign deep fibromatous tumours originating from fascia and muscle aponeurosis with an infiltrating growth. They arise primarily in the extremities, trunk wall and abdominal cavity.[1] This type of tumour is aggressive locally and can invade other surrounding tissues and adjacent organs although it generally cannot metastasise or seldom spread to distant parts of the body. Desmoid tumour is also referred to as 'aggressive fibromatosis' and the clinical course of desmoid tumour is unpredictable and some spontaneous regression has been observed. This nature of desmoid tumour results in a 'wait and see' approach which is been recently proposed.[2]
| Incidence Of Desmoid Tumour | | |
Desmoid tumors are found in all age groups. However, they are frequently occurring between the ages of 10–40 years. They are more often seen in women after childbirth and the ratio of frequency for males and females is 2:1. However in children, the gender ratio is equal. There are about 2–4 new cases found per million population annually globally, in which 16% of the incidence of desmoid lesions is associated with familial adenomatous polyp (FAP) compared to 84% of sporadic lesions.[3] These tumours are more aggressive in younger patients, with recurrence rates up to 87%.[3]
| Cause Of Desmoid Tumours | | |
The cause of desmoid tumours has not been well defined. The tumours may be sporadic resulting from gene mutation called somatic mutations or occur during the lifetime of a healthy individual. It can also be a manifestation of hereditary FAP which is an autosomal dominant disorder. FAP is a familial cancer predisposition syndrome which, if left untreated, results in colorectal cancer. Up to 32% of patients with FAP develop desmoid tumours in their lifetime.[1]
| Risk Factors of Desmoid Tumours | | |
The following are the common risk factors:[4]
- Family history of FAP
- Gardner syndrome
- Repeated irritation to the body part which includes surgical trauma, trauma
- Women with high oestrogen levels
- Trauma such as laparotomy, abdominal trauma
- Oral contraceptive use
- Pregnancy.[5]
| Pathophysiology of Desmoid Tumours | | |
The genes implicated in the cause of desmoid tumours are the Catenin Beta (CTNNB1) gene and the adenomatous polyposis coli (APC) gene. CTNNB1 gene is responsible for the provision of instructing the brain in the production of Beta-Catenin protein. The APC gene on the other hand produces a protein that is responsible for regulating the levels of beta-catenin in the cells. The mutational status of Beta-catenin gene is presumed to affect the tumerous activity of the cells.[6]
| Clinical Manifestations | | |
Desmoid tumours can develop in any part of the body although it is more common to develop in the shoulder girdle and in the abdominal wall. The signs and symptoms of Desmoid tumour depend on the site, location, and spread of the tumour. Desmoid tumour is basically divided into three classifications according to the area of development and includes the following[5]
Intra-abdominal desmoid
They are soft-tissue tumours that generally develop in the root of the mesentery. They cause severe pain, rectal bleeding, compression of the kidney or ureters, rupture of the intestines. However, intra-abdominal desmoids remains asymptomatic until it increases in size and the infiltration causes compression of the blood vessels and other adjacent vital organs.
Abdominal desmoids
They are superficial desmoids that develop in the abdominal wall. A painless and enlarging mass and is rock hard upon palpation. Painless or slightly painful lump is the usual manifestation of superficial desmoids.
Extra-abdominal desmoids
These are superficial desmoids that develop in the areas of pelvic girdles, upper and lower arms. They are rather rare and usually manifest with a gradual swelling of the leg. The tumour is smooth, firm, movable, growing lump which can be palpable and is associated with pain which can be mild to moderate with numbness and tingling limiting range of movement.
| Diagnosis | | |
Fine-needle aspiration cytology -fine-needle aspiration for cytolological test-It is the most conclusive diagnostic test.[7]
Radiological imaging
- Magnetic resonance imaging (MRI)-MRI with soft-tissue contrast allows accurate depiction of extra-abdominal desmoids tumour's relationship with adjacent structures. It is best suited for optimum evaluation of extra-abdominal desmoids
- Computed tomography (CT)-shows a soft-tissue mass of variable attenuation and enhancement[5]
- Ultrasound - Desmoid tumours appear as hypoechoic soft-tissue masses with variable vascularity.[5]
Electron microscopy
The spindle cells of desmoid tumours appear to be myofibroblasts on electron microscopic examination. This finding represents an abnormal proliferation of myofibroblasts. Electron microscopy is performed in addition to the biopsy for obtaining further clarity and confirmation of the diagnosis.
Beta catenin protein
Immunohistochemical stains can be done looking for nuclear accumulation of beta-catenin, a protein that is affected by the genetic mutations often found in desmoid tumours.[7]
| Management | | |
For optimum management of desmoid tumours, a multidisciplinary team approach should be tailored to the individual patient. Stable asymptomatic desmoids can be observed.[8] Treatment is necessary for symptomatic desmoids, especially those with mass effect on critical structures. Treatment choice is dictated by anatomic considerations. While the tumour has no ability to metastasise, surgery is often the choice and initial step of treatment. The tumour can be treated with surgery, chemotherapy, radiation, hormone therapy and other medications. In some cases, chemotherapy may not be necessary as the tumour has no metastatic potential. For most cases of desmoid tumour, a combination of surgery, radiation therapy, hormone therapy and medications are used to successfully treat the patient.
Surgery
The conventional treatment of choice for symptomatic resectable desmoids is the surgical resection with a wide margin. Recurrence is common (19%–77%) and more frequent with extraabdominal desmoids (30%–50%) than intra-abdominal desmoids (15%–30%). More often, resection may not be possible because of tumour's association with vital structures. In these cases, systemic therapy with or without surgical excision should be considered. Mesenteric desmoids associated with FAP or Gardner's syndrome are often unresectable or lead to significant morbidity, including perioperative haemorrhage, short-bowel syndrome, intestinal ischaemia, obstruction or fistula formation. Hence, in such cases, conservative management has been recommended whenever possible, using analgesia and minimally invasive therapy as required. Recently, there has been an increasing shift towards conservative management of desmoids.[5]
Radiation therapy
Radiation therapy (RT) may be used as a treatment for recurrent disease or as primary therapy to avoid mutilating surgical resection. It is used alone in situations where surgery isn't possible. Radiation has been reported to be comparable to surgery and is also useful as an adjuvant treatment to reduce the risk of local recurrence.[9]
Chemotherapy
If tumour cannot be removed because of the size or location as in the case of recurrent extra-abdominal desmoid tumours in which surgery is contraindicated, some types of chemotherapy are used to reduce tumour size. Systemic treatment options include cytotoxic agents, such as anthracyclines, molecular-targeted agents, such as imatinib; interferon; and anti-oestrogen hormonal therapy such as tamoxifen. Systemic agents such as anthracyclines and anti-oestrogen treatment has been reported to have a higher radiologic response.[10] The drugs usually administered are doxorubicin, dacarbazine and carboplation.
Anti-inflammatory drugs
Anti-inflammatory drugs is given to help manage pain, swelling and can also cause the tumour to slowly shrink. Drugs such as imatinib and non-steroidal anti-inflammatory drugs are being prescribed by few clinicians to treat desmoid tumours. Imatinib has been shown to effectively treat desmoid tumours that cannot be safely removed via surgical means.
Novel molecular-targeted therapies
Kinases are regulators of cell growth, differentiation, and motility. Since these processes are deregulated in tumours cells, a new class of drugs called receptor kinase inhibitors has been developed. Gleevec and Sorafenib are two kinase inhibitors that are being used in the treatment of desmoid tumours.[7]
Heating or freezing tumour tissue
Ablation therapy uses extreme heat (radiofrequency ablation) or cold (cryoablation) to kill tumour cells. In a procedure of this type, a special probe is inserted through the skin and into the tumour. To ablate tissue during cryoablation, the cryoprobe freezes the area, causing the surrounding tissue to die. During radiofrequency ablation, electromagnetic energy is used to heat the tissue to high temperatures and results in cell death.[7]
Monitoring
After surgery, MRI may be useful for monitoring recurrence. Positron-emission tomography scans is also growing in popularity for monitoring the recurrence of desmoid tumours.
Watch and wait policy
Desmoid tumours do not metastasse and can be monitored closely for growth and because treatment with surgery and/or RT and chemotherapy can cause significant morbidity and even mortality, patients with asymptomatic or minimally symptomatic disease and with stable appearance on screening modalities may appropriately be treated with a period of watchful waiting.[7]
Hormone therapy
Hormone such as oestrogen is found to increase the growth of desmoids, anti-oestrogen and prostaglandin inhibitors are used.
| Prognosis | | |
The prognosis for desmoid tumour depends on the behaviour of the tumour, whether it is growing aggressively or slowly or whether it is truly benign or truly malignant. The compression of involved organ also accounts for the prognosis of the tumours.
The risk for local recurrence is as high as 19%–77%.[4]
| Complications | | |
Complications of desmoid tumours result from their locally aggressive nature, leading to compression and invasion of adjacent structures:[5]
- Intra-abdominal desmoids, especially those associated with FAP or Gardner syndrome, are more infiltrative and may cause intestinal or ureteral obstruction or encase the mesenteric vessel
- Pelvic desmoids can infiltrate the urinary bladder or may cause hydrosalpinx
- Extra-abdominal desmoids may compress and encase adjacent structures, including vessels or nerves
- Chest wall desmoids can invade the pleura
- Desmoids rarely undergo abscess formation which may require surgical intervention or percutaneous drainage
- Large desmoid tumours may undergo cystic or mucoid degeneration.
| Case Report | | |
Mr. K A 35-year-old man diagnosed to have FAP, underwent laparoscopic total colectomy with an ileal pouch and rectal anastomosis and covering loop ileostomy followed by ileostomy closure. After a year, he developed abdominal and back pain due to mesenteric abscess for which he underwent laparotomy and drainage of abscess. In view of his persistent symptoms, he underwent laparotomy and drainage of mesenteric abscess twice within a period of 7 months. After 2 years of his primary surgery, his ileorectal pouch was dismantled and he underwent pouch excision with permanent end ileostomy.
When he came for his follow-up one and a half years later, CT abdomen and pelvis revealed mesenteric desmoid tumour and he was started on tamoxifen. After a year, he presented with complaints of recurrent parastomal abscesses and underwent abscess drainage with corrugate drain placement. The drain was noted to have faeculant discharge. An ill-defined, firm, non-tender mass was noted in the parastomal region inferior to the ileostomy. The mass did not move with respiration. Another similar mass was noted in the umbilical region. His CT abdomen and pelvis revealed mesenteric mass involving anterior abdominal wall, loop of proximal ileum and right abdominal wall near the stoma site. Another speculated soft tissue lesion suggestive of desmoid tumour was noted encasing the right ureter at the level of pelvis inlet causing moderate hydronephrosis.
An exploratory laparotomy was planned and executed. There was a large desmoid in the small bowel mesentery infiltrating mid and lower abdominal wall. Another mass was felt in mesentery of the end ileostomy. There were interloop adhesions of jejunum, liver and stomach. The ileum was involved in the desmoid and could not be freed. There was an inadvertent 0.5 mm rent in ileum, which was repaired with 3/1 polydioxanone suture. On table enteroscopy was attempted. Acute kink precluded progress beyond 30 cm. In view of bowel that was not free and certainty of doing more harm than good, resection of the tumour was abandoned.
Postoperatively, he continued to have feculent discharge from the parastomal fistula mixed with altered blood. He also complained of pain in the abdomen associated with vomiting which was nonbilious in nature. He was managed conservatively with adequate fluids, antiemetics, proton-pump inhibitors and analgesic with which he improved. He was taught how to manage the fistula with the help of a wound manager and was discharged from hospital.
| Nursing Care | | |
The specific nursing perspectives pertaining to fistula and stoma management for a patient with desmoid tumour is presented here using nursing process approach.
1. Nursing Diagnosis
Acute pain related to surgery done and presence of parastomal abscess.
Expected outcome
Pain is reduced as evidenced by verbalisation of decrease in pain score and relaxed facial expression.
Interventions
- Assessed the pain score and characteristics of pain
- Assessed the aggravating factor and alleviating factor
- Administered narcotic analgesic (Injection bupivaccaine and Injection fentynl) via epidural infusion as per the order
- Provided comfortable position
- Provided diversional therapy
- Provided comfort devices
- Advised him to splint the operated site while getting up, sitting or walking
- Pain education.
Evaluation
Pain was reduced as evidenced by verbalisation of decrease in pain score and relaxed facial expression.
2. Nursing Diagnosis
Imbalanced nutrition: less than body requirement related to vomiting and avoidance of certain food that may cause discomfort due to presence of stoma.
Expected outcome
Nutritional status will be enhanced as evidenced by no vomiting or further decline in strength, weight loss.
Interventions
- Assessed the nutritional status of the patient
- Assessed for the frequency, relieving and aggravating factors for nausea and vomiting
- Assessed for the signs and symptoms of dehydration
- Served food according to their likes and dislikes
- Provided small, frequent meals
- Provided clean environment
- Avoided painful procedures before the meal time
- Administered antiemetics (Injection Emeset 4 mg/every 8th hourly) half an hour before the food
- Assessed the nutritional requirement
- Performed 24 h dietary recall
- Monitored weight regularly
- Monitored intake and output ratio
- Teaching on dietary modifications to be made for a person on ileostomy was provided
- Instructions on emptying the stoma bag were given.
Evaluation
Nutritional status was enhanced as evidenced by no vomiting or further decline in strength.
3. Nursing Diagnosis
Disturbed body image related to loss of control of bowel function secondary to stoma and presence of enterocutaneous fistula.
Expected outcome
He will be able to verbalse acceptance of self and adaptation to altered body image and verbalise understanding of changes.
Interventions
- Encouraged him to verbalise his feelings about stoma and fistula
- Acknowledged his feelings of depression, anger and grief as normal feelings when adjusting to changes in his body
- Explored his strength and resources available to support him
- Allowed him and others to understand the changes that his body has undergone
- Encouraged him to make his own decisions, participate in plan of care, accept both inadequacies and strengths
- Encouraged him to continue same personal care routine that was followed before the change in body image
- Taught all aspects of care by involving him and his wife in self-care as soon as possible
- Provided printed material about information on stoma
- Introduced him to another patient with a similar condition but has accepted the change in his body image
- Ensured family participation in care to enhance support.
Evaluation
He verbalised his feelings about his stoma and started to deal with it constructively. He was able to take care of his stoma and fistula.
4. Nursing Diagnosis
Ineffective individual coping related to presence of stoma and fistula and associated social stigma.
Expected outcome
Coping is enhanced as evidenced by remaining calm and acknowledgement of his own coping abilities.
Interventions
- Provided an atmosphere of acceptance
- Provided factual information concerning the treatment and prognosis of his disease
- Encouraged verbalisation of feelings, perceptions and fears
- Observed the degree of family support
- Determined the barriers to using support systems
- Involved his wife in the care and planning
- Encouraged him to identify his own strengths and abilities
- Encouraged him use of stress management skills such as relaxation techniques, guided imagery and deep breathing exercises
- Provide psychological support and spiritual support.
Evaluation
His coping outcome was partly met. Though he was receptive to the psychological support offered by the stoma therapist, he was withdrawn and expressed anger on his wife. He was not able to accept his poor prognosis.
5. Nursing Diagnosis
Risk for impaired skin integrity related to irritation of the peristomal skin by the effluent from the stoma and fistula.
Expected outcome
Normal skin integrity is maintained as evidenced by absence of peristomal and perifistula skin excoriation.
Interventions
- Inspected the stoma and the peristomal skin with each pouch change
- Measured stoma during each pouch change and the size of the appliance was altered to ensure proper fit, so that the effluent (output) is collected into the bag and contact with the skin is prevented
- Used a transparent, odour proof drainable pouch which allows easy observation of the stoma without the necessity of removing the pouch and irritating the skin
- Used a pouch with hydrocolloid skin barrier which protects the skin from the irritating nature of the effluent
- Emptied pouch whenever it was filled three fourth thereby avoiding leakage from the pouch
- Provided information about signs and symptoms of irritated or inflamed skin
- Taught him to clean the peristomal region gently with water and pat dry
- Supported peristomal skin while gently removing the appliance which prevented tissue damage
- Counselled him on diet to maintain the consistency of stools
- Changed appliances as and when it was necessary
- Demonstrated him how to pouch the enterocutaneous fistula and ensured that he was able to perform fistula care independently.
Evaluation
The integrity of the skin was maintained as evidenced by appearance of normal skin around stoma and fistula.
6. Nursing Diagnosis
Risk for injury: stomal necrosis, haemorrhage, retraction, prolapsed and intestinal obstruction related to lack of blood supply, increased intra-abdominal pressure.
Expected outcome
Patient will not suffer injury as evidenced by normal appearing and functioning stoma.
Interventions
- Applied a transparent drainable pouch in the post-operative period which helps in observation of the appearance of the stoma
- Monitored the colour and the moisture of the stoma every 4th hourly
- Observed the stoma for pale, dusky or blue appearance which may indicate venous congestion or poor blood supply resulting in stomal necrosis
- Ensured that there is no pressure over the stoma that could interfere with the circulation
- Observed the stoma for bleeding, retraction, prolapse and stenosis
- Assessed for anorexia, abdominal cramps, nil ileostomy drainage, foul, brown, watery discharge in the pouch or visible peristalsis which indicate intestinal obstruction due to obstruction of the lumen, adhesions or stomal oedema.
Evaluation
Patient did not suffer injury as evidenced by normal functioning stoma. Stoma remained pink, moist with no bleeding, retraction, prolapse or stenosis.
7. Nursing Diagnosis
Ineffective health management related to stoma and fistula care and home care.
Expected outcome
Client demonstrates ability to empty and change pouch and acquires information about diet, activity, clothing, odour, purchase of the equipment.
Interventions
- Provided psychomotor teaching during first and subsequent applications of the pouch
- Included his wife during the teaching process in the early period although the goal is for the patient to become independent in self-care
- Gradually transferred responsibility for pouch emptying and changing to the client
- Ensured supervised return demonstration of pouch change
- Helped him to choose the best pouch according to his needs and economic status
- Instructed him balanced diet, special care in chewing high fibre foods (vegetables, coconut, nuts, popcorn), increased fluid intake during hot climate to avoid dehydration
- Discussed odour control and acknowledged that fear of odour can affect social functioning. Advised to minimise odour causing diet like green leafy vegetables, eggs, fish and onions. Gave information about pouch deodorants (charcoal, tissue paper soaked in Listerine mouth wash)
- Informed him that activity should not be affected and no special clothing or alteration in existing clothing are necessitated by the presence of stoma
- Gave special instructions regarding prescriptions and over the counter medications enteric coated tablets, multilayer tablets, time release capsules may not be absorbed in the small intestine
- Showed him pictures of possible stomal complications and advised to get medical help when needed
- Instructed him to avoid rough contact sports (to prevent stomal injury) and heavy weight lifting (to prevent increased intra-abdominal pressure that causes prolapsed and parastomal hernia)
- Discussed the availability of ostomy support groups, and where to purchase pouch
- Instructed him to maintain contact with enterostomal therapist for follow-up and observation and problem solving.
Evaluation
He demonstrated ability to empty and change pouch and acquired information about diet, activity, clothing, odour, drugs, purchase of the equipment.
| Conclusion | | |
Desmoid tumour is a challenging clinical condition with locally aggressive behaviour and a strong tendency for recurrence. Patients with asymptomatic or minimally symptomatic disease and with stable appearance on screening modalities may appropriately be treated with a period of watchful waiting. However, some patients have advanced disease which is not surgically resectable. Conservative management has been recommended in such cases whenever possible, using analgesia and minimally invasive therapy as needed. Nurses play a very important role in supporting and educating patients to adopt a healthy lifestyle to remain free of symptoms. Clients and family will need social and psychological support.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
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