Palliative Surgery for Metastatic Fungating Phyllodes Tumors: A Series of Two Cases



 

Aakanksha Goel, MBBS, MS; Rahul Insa, MBBS, MS;
Manish Kumar Gaur, MBBS, MS;
Pankaj Kumar Garg, MS, DNB, MCh

Perm J 2018;22:17-100 [Full Citation]

https://doi.org/10.7812/TPP/17-100
E-pub: 07/19/2018

Abstract

Introduction: Local treatment of metastatic cystosarcoma phyllodes is classically viewed with skepticism because it does not provide survival benefit. When these advanced tumors ulcerate, they reduce quality of life dramatically because of associated pain, infection, malodor, massive discharge, and bleeding.
Case Presentations: Two patients with metastatic cystosarcoma phyllodes presented to our hospital with recurrent disease featuring foul-smelling, ulcerated chest wall masses that caused physical pain and social exclusion. The first patient underwent radical chest wall resection with pedicled lattisimus dorsi flap reconstruction. The second patient underwent wide local excision with split-thickness skin grafting. There was significant improvement in the quality of life for these patients after the operations.
Discussion: Though both patients finally succumbed to progressive metastatic disease, palliative resection allowed them to have good social and family support to the end. Palliative surgery plays an important role in alleviating suffering of patients with metastatic fungating cystosarcoma phyllodes and has the potential to improve their quality of life significantly.

Introduction

Phyllodes tumor, or cystosarcoma phyllodes, is a fibroepithelial tumor of the breast with a diverse range of biological behavior. These tumors may appear as benign fibroadenoma-like lesions, locally recurrent aggressive tumors, or widely metastatic malignant forms. When these advanced tumors ulcerate, they reduce quality of life dramatically because of associated pain, infection, malodor, profuse discharge, and bleeding. In patients with distant metastasis, chemotherapy is considered the first-line therapy on the basis of principles of soft-tissue sarcoma.1 Local surgical treatment of metastatic phyllodes is classically viewed with skepticism because it does not provide survival benefit and is sparsely reported in the literature.2 We present our experience with two cases of recurrent metastatic phyllodes tumors, our surgical management of which significantly ameliorated the patients’ symptoms.

Case Presentation

The following two cases of metastatic recurrent cystosarcoma phyllodes with ulceroproliferative chest wall masses were operated on with palliative intent in the surgery unit of a tertiary care hospital in Delhi, India. Both patients gave informed consent.

Case 1

A 28-year-old woman presented to our hospital with a large fungating chest wall mass (Figure 1). She had undergone a margin-negative left mastectomy for a large phyllodes tumor and received adjuvant radiotherapy 8 months earlier. At presentation, the new growth was 15 cm × 13.5 cm in size and fixed to the chest wall. She reported disabling pain on movement of the shoulder joint and foul odor of the discharge. An incisional biopsy of the breast mass confirmed the recurrence of cystosarcoma phyllodes. Contrast-enhanced computed tomography of the chest revealed multiple metastatic lung nodules.

The patient had been married barely 2 months before her first surgery and was facing the stigma of being impure and contagious. Her parents-in-law felt cheated for having been given a “less-than-perfect” daughter-in-law, and her society shunned her for being a disfigured, foul-smelling woman. Moreover, her relationship with her husband was increasingly becoming strained; he was burdened by the responsibility to take care of her, to financially support the family, and to manage the household, and she was suffering from mood swings, depression, and helplessness. Her Hamilton Depression Rating Scale (HDRS) score was 24. Surgical palliation was decided on, keeping in mind the patient’s good performance status and absence of comorbid conditions.

Our patient underwent radical resection of the chest wall tumor with latissimus dorsi flap-based chest wall reconstruction. Postoperatively, she expressed her extreme happiness at getting rid of the foul-smelling tumor. Her HDRS score decreased substantially to three. Subsequently, she was referred to the Medical Oncology Department for systemic chemotherapy. However, her disease progressed and she succumbed to her illness six months after surgery. Figure 2 presents a timeline of her case.

Case 2

A 40-year-old postmenopausal woman who had undergone right radical mastectomy for a locally advanced cystosarcoma phyllodes tumor 6 months earlier presented to our hospital with a large right anterior chest wall fungating mass. The mass measured 28 cm × 24.5 cm and was abutting the right axillary vein (Figure 3). The patient was unable to move her arm, and the large tumor caused gross disfigurement, persistent foul odor, and recurrent bleeding, leading to social exclusion for her. An incisional biopsy of the chest wall mass confirmed the recurrence of the phyllodes tumor. A contrast-enhanced computed tomography scan of the chest revealed multiple lung metastases.

The patient and her family expressed their desire for a palliative resection to improve her quality of life despite knowing and understanding well that it would not improve her survival. Her HDRS score was 21. The tumor was resected with a palliative intent and the defect was covered with a split skin graft. She did well postoperatively, and her quality of life improved significantly. Her HDRS score also decreased substantially to 3. Although she was referred to the Medical Oncology Department, she declined chemotherapy. She went back to her village where she succumbed to her progressive lung metastasis 5 months after surgery. Figure 4 presents a timeline of this case.

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Discussion

Phyllodes tumors of the breast are biphasic tumors, classified into benign, borderline, and malignant histological types.3 The name “cystosarcoma phyllodes” is derived from the characteristic leaf-like architecture of the tumor on microscopic examination, which contains varying degrees of epithelial and mesenchymal elements.4 Malignant phyllodes tumors can recur as well as metastasize and have a uniformly poor prognosis. Less than 5% of all phyllodes tumors metastasize.5 Although margin-negative surgical resection is the treatment of choice for localized benign and malignant disease, the role of surgery for metastatic phyllodes remains unexplored, primarily owing to lack of survival benefit. The usual survival for metastatic phyllodes is less than 2 years.2

Patients with large fungating malignant phyllodes with metastasis experience poor quality of life owing to foul-smelling discharge, pain, bleeding, disfigurement, and limitation of movement. Adding to the physical symptoms are the psychosocial problems, especially in the setting of low-income families in developing countries who may have a lack of awareness and may have superstitions regarding diseases.

In our first case, after discharge, the patient was able to go home and was received favorably by the family and her society. She was able to recognize the efforts of her husband in trying to make her feel well. Despite knowledge of her advanced disease, she felt happy after a long period of depression. She was able to take care of herself until she succumbed to her disease. The second woman, too, postoperatively was able to take better care of her children and was able to move in her social circle without the stigma of disfigurement. Both women were able to accept their impending fate by embracing spirituality. The HDRS score decreased substantially in both patients, reflecting the success of palliative surgery.

Although both patients succumbed to the aggressive disease, there was remarkable improvement in their quality of life in their last days. Thus, in the absence of clinical trials to assess the best treatment regimen for metastatic phyllodes and only anecdotal experience with systemic therapy and radiotherapy, these two cases reinforce the role of surgical palliation in well-selected patients. Although it was initially seen with a pessimistic view, palliative surgery is increasingly being recognized as an important and inevitable component of comprehensive cancer care, which may be adopted for phyllodes tumors as well.6 Palliative surgery empowers patients to maintain independent function, thereby minimizing the burden of care on family members, albeit for a limited time.

The current lack of prospective studies in the literature to validate palliative surgery for phyllodes tumors, which is the result of ethical considerations such as patients’ vulnerability and the moral implication of doing research on terminally ill patients, needs to be negated with the use of alternative outcome measures to benefit this patient population. However, costs and resources associated with provision of palliative surgery need to be anticipated and balanced with the ultimate outcome. It also needs to be highlighted that practitioners must be cautious in explaining the noncurative nature of the palliative surgical procedures so as to provide realistic treatment goals to these patients and their caregivers. Although the evidence generated by this case series of two patients is limited, it further demonstrates that palliative surgery is an optimal and legitimate treatment approach for metastatic fungating phyllodes tumors.

Disclosure Statement

The author(s) have no conflicts of interest to disclose.

Acknowledgments

Mary Corrado, ELS, provided editorial assistance.

How to Cite this Article

Goel A, Insa R, Gaur MK, Garg PK. Palliative surgery for metastatic fungating phyllodes tumors: A series of two cases. Perm J 2018;22:17-100. DOI: https://doi.org/10.7812/TPP/17-100

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