Treatment for Gorham-Stout Disease Involving the Spinal Column: A Narrative Review

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www.krspine.org

Treatment for Gorham-Stout Disease Involving the Spinal Column: A Narrative Review

Sam Yeol Chang, M.D., Hyoungmin Kim, M.D., Ph.D., Bong-Soon Chang, M.D., Choon-Ki Lee, M.D.

J Korean Soc Spine Surg 2021 Jun;28(2):55-62.

Originally published online June 30, 2021;

https://doi.org/10.4184/jkss.2021.28.2.55

Korean Society of Spine Surgery

Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea, 88, Olympic-ro 43-gil, Songpa-gu, Seoul 05505, Korea

Tel: +82-2-831-3413 Fax: +82-2-831-3414

The online version of this article, along with updated information and services, is located on the World Wide Web at:

http://www.krspine.org/DOIx.php?id=10.4184/jkss.2021.28.2.55

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://

creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Journal of Korean Society of

Spine Surgery

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Treatment for Gorham-Stout Disease Involving the Spinal Column: A Narrative Review

Sam Yeol Chang, M.D., Hyoungmin Kim, M.D., Ph.D., Bong-Soon Chang, M.D., Choon-Ki Lee, M.D.

^* Department of Orthopedic Surgery, College of Medicine, Seoul National University, Seoul, Korea

*Department of Orthopedic Surgery, Chamjoeun Hospital, Gwangju, Gyeonggi-do, Korea

Study Design: Narrative review.

Objectives: To present up-to-date evidence on the treatment of Gorham-Stout disease (GSD) involving the spinal column.

Summary of Literature Review: Gorham-Stout disease (GSD) is a rare disease that is characterized by progressive bone resorption associated with vascular or lymphatic angiomatosis. Although various treatment options have been described and investigated, there is no consensus on the appropriate treatment for GSD.

Materials and Methods: Review of the associated literature and latest research.

Results: The available treatment options for spinal GSD are systemic agents (including interferon-alpha, bisphosphonate, denosumab, and sirolimus), radiotherapy, surgical treatment, and percutaneous vertebroplasty.

Conclusions: Treatment for spinal GSD should be case-specific and involve multiple modalities, including systemic medications, radiotherapy, and surgical treatment.

Key words: Gorham-Stout disease, Denosumab, Sirolimus, Radiotherapy, Vertebroplasty

Received: January 6, 2021 Revised: January 14, 2021 Accepted: April 27, 2021 Published Online: June 30, 2021

Corresponding author: Hyoungmin Kim, M.D., Ph.D.

ORCID ID: Sam Yeol Chang: https://orcid.org/0000-0003-4152-687X Hyoungmin Kim: https://orcid.org/0000-0002-4500-9653 Bong-Soon Chang: https://orcid.org/0000-0002-8992-2559 Department of Orthopedic Surgery, Seoul National University Hospital, 101 Daehang-ro, Jongno-gu, Seoul, Korea

TEL: +82-2-2072-0357, FAX: +82-2-764-2718 E-mail: [email protected]

Introduction: The Gorham-Stout Disease

Gorham-Stout disease (GSD) is a rare disease that is characterized by a progressive bone resorption associated with vascular or lymphatic angiomatosis. This rare condition has been described in the literature using various names, such as “disappearing bone disease” and “vanishing bone disease”.¹⁾ Since Gorham and Stout reviewed 24 cases from the literature in 1955, it has been referred to as Gorham’s disease or GSD.²⁾ GSD is also classified as one (type IV) of five idiopathic osteolysis syndromes.³⁾ Although, the underlying pathophysiology remains unclear, uncontrolled proliferation of lymphatic vessels and increased osteoclastic activity seem to cause progressive osteolysis.⁴⁾

The clinical manifestations of a patient with GSD depends on the location of the disease. GSD commonly involves the skull, clavicle, ribs, spine, pelvic girdle, and femur.⁵⁾ Patients with spine involvement can experience cerebrospinal fluid (CSF) leakage and paralysis due

to spinal instability. Spontaneous CSF leakage due to increased permeability of the dura mater can lead to intracranial hypotension and subsequent brainstem herniation (Fig. 1).^6,7) GSD involving the rib cage or thoracic spine can cause recurrent pleural effusion or chylothorax, which can be often intractable and fatal.⁸⁾ As for the diagnosis of GSD, Heffez et al suggested the following 8 diagnostic criteria of Gorham-Stout disease:

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Sam Yeol Chang et al Volume 28 • Number 2 • June 30 2021

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(1) positive biopsy findings in terms of angiomatous tissue presence; (2) absence of cellular atypia; (3) minimal or no osteoclastic response and absence of dystrophic calcifications; (4) evidence of local bone progressive resorption; (5) non-expansive, non-ulcerative lesion; (6) absence of visceral involvement; (7) osteolytic radiographic pattern; and (8) negative hereditary, metabolic, neoplastic, immunologic and infectious etiology.⁹⁾

Currently, the literature investigating GSD is limited to case reports or small case series. To date, there are about 300 reported cases of GSD in the literature, whereas GSD with spine involvement comprises less than 100 cases.¹⁰⁾ Because of its rarity, there is no consensus on treatment strategies for GSD. In this review, we present updated evidence focused on the treatment of GSD involving the spinal column.

Treatment for Gorham-Stout Disease

Various treatment options have been suggested and investigated for GSD treatment in the literature, including systemic agents, radiotherapy, and surgical resection and stabilization. However, there is no consensus on the standard treatment for GSD. Therefore, treating a GSD patient requires an interdisciplinary approach that utilizes multiple treatment modalities. Treatment for GSD should also be case-specific because the clinical manifestation varies significantly between GSD patients based on the location and extent of the disease. The available treatment

options for GSD are discussed in this review.

1. Systemic agents

Various agents have been suggested, including vitamin D, interferon (IFN) alpha, calcium, calcitonin, heparin, anticoagulants, anti-resorptive agents (bisphosphonate and denosumab), bevacizumab, and most recently, sirolimus. However, consensus is lacking regarding which is the most effective systemic treatment for GSD. In this section, evidence from the literature regarding IFN alpha, bisphosphonate, denosumab, and sirolimus is presented.

Several authors have reported on GSD cases that were successfully managed with IFN alpha.^11-13) IFN alpha was often used with other treatment modalities such as oral clodronate,¹¹⁾ steroid pulse therapy,¹²⁾ vertebroplasty,¹⁴⁾ and surgical resection.¹³⁾ IFN alpha was considered in GSD patients, especially in extensive cases, because of the antiangiogenic property of IFN. Although there is no definite guideline regarding when to stop IFN treatment in GSD patients, patients who received IFN treatment showed clinical improvement and maintenance after 10 to 20 months of treatment.^11,12) The dosage of IFN used in the literature was 1,000,000 to 4,000,000 units/day for induction and 1,500,000 units/week for maintenance.

Previous studies did not report any adverse events associated with IFN alpha treatment; however, common side effects of IFN alpha including flu-like symptoms, nausea, fatigue, and hematological toxicity should be considered when using IFN alpha.

Osteoclast activity is increased in GSD patients causing osteolysis, although the exact mechanism is unclear.

Therefore, it is reasonable to consider bisphosphonate, an anti-resorptive agent, as a treatment option for GSD.

In the literature, several authors reported successful GSD management using bisphosphate, which slowed down disease progression and reduced physical disability.^5,15,16) Like other systemic agents, bisphosphate was also utilized with other GSD treatments in most case reports. Although long-term bisphosphonate treatment for up to 17 years has been reported,⁵⁾ there is no consensus on the duration of bisphosphonate use for GSD, especially considering the long-term side effects of bisphosphonates, such as atypical fractures and osteonecrosis of the jaw.

Fig. 1. (A) T2-weighted magnetic resonance imaging (MRI) of an 18-year- old male patient with extensive Gorham-Stout disease in the spinal column. Severe spinal cord compression is observed at the apex of thoraco- lumbar kyphosis. (B) T1-weighted brain MRI shows brainstem herniation due to intracranial hypotension.

A B

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Denosumab, another anti-resorptive agent, is a human monoclonal antibody to the receptor activator of nuclear factor-kB ligand (RANKL), which decreases bone resorption in patients with osteoporosis and skeletal metastasis. To the best of our knowledge, there are three case reports in the literature in which denosumab was used to control GSD in the skull base (1 case),¹⁷⁾ mandible (1 case),¹⁸⁾ and shoulder girdles (2 cases).¹⁹⁾ Denosumab 60–120 mg every 6 months showed mixed results in these reports. Therefore, more evidence is necessary to clarify the effectiveness and safety of long-term denosumab treatment, especially in cases of spinal GSD.

Sirolimus, an mTOR (mechanistic target of rapamycin) inhibitor, is increasingly being used in various lymphatic disorders, including GSD, especially for refractory cases to other treatments (Fig. 2). mTOR regulates cell proliferation and promotes angiogenesis, whereas the mTOR inhibitor has an antiangiogenic effect in lymphatic disorders.²⁰⁾ In a phase-II clinical trial of sirolimus that investigated 61 patients with lymphatic and vascular disorders, including 3 GSD cases, 93.0% of patients showed a treatment response at the end of sixth treatment course.²¹⁾ In this study, two patients required dose reduction and another two patients were taken off the drug because of grade 3 or higher adverse events.

Regarding spinal GSD, sirolimus has been used as neoadjuvant therapy before surgical resection and reconstruction of a spinal GSD lesion to minimize intraoperative and postoperative lymphatic drainage and prevent related complications. In a case reported by Mo et al., 7 weeks of sirolimus treatment prior to surgery prevented lymphatic drainage in the surgical field.⁹⁾ However, the adequate duration of preoperative sirolimus treatment for preventing lymphatic drainage has not been determined.

Systemic agents for GSD treatment are generally used in combination or sequentially. Although available evidence is limited because of the rarity of the disease, combining agents that inhibit the proliferation of lymphatic vessels (sirolimus) and bone resorption (bisphosphonate, denosumab) is a promising approach because these drugs act on different pathophysiological stages of GSD. Future studies should verify the effectiveness of this combined approach and determine the appropriate treatment protocol, including the dosage and duration of the medications. Surgeons have also used systemic medications to control GSD before surgical treatment.^9,13,22) The clinical and pathological effects of neoadjuvant systemic treatment should also be investigated in future studies.

Fig. 2. (A) Chest X-ray of a 13-year-old male patient shows Gorham-Stout disease (GSD) involving multiple ribs on the right side with pleural effusion. (B) Despite 4 years of treatment, including thoracic duct ligation, bisphosphonate, interferon-alpha, and systemic steroid administration, the disease progressed, leading to destruction of the right lung. At the age of 17, the patient started sirolimus for GSD. (C) After 1 year of sirolimus treatment, the disease progression was finally controlled. The patient is currently on sirolimus (5 mg daily) and under close surveillance for the progression of thoracic scoliosis.

A B C

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2. Radiotherapy

Radiotherapy is used in GSD patients to control disease progression and reduce pain with varying success rates.²³⁾ In 2011, the German Cooperative Group on Radiotherapy for Benign Diseases published a national patterns-of-care study that investigated the value of radiotherapy in GSD treatment.²⁴⁾ To the best of our knowledge, this study is by far the largest single study in the literature as it included 10 GSD patients from 8 institutions. After a median follow-up of 42 months, the authors reported that disease progression was prevented in 8 of 10 cases following radiotherapy with a total dose of 30 to 45 Gy. They also reviewed 38 previously published studies and found that 77.3% (34/44) of patients that received radiotherapy showed stable or regressive disease. However, it is unclear whether radiotherapy can be considered a definitive treatment modality for inducing disease remission in GSD.

3. Surgical treatment

In the literature, many authors have reported cases in which surgery was performed for spinal GSD.9,22,25,26)

However, surgery for spinal GSD is often challenging because, in many cases, the disease can be extensive, resulting in a highly unstable spine with minimal bone stalk for fusion and reconstruction. In some cases, spinal reconstruction is infeasible because of massive osteolysis.²⁷⁾ During surgery, lymphatic fluid can leak into the surgical field and interfere with surgical procedures.⁹⁾ For these reasons, previous studies have reported a high revision rate after spinal GSD surgery. Tateda et al. reviewed all spinal GSD surgical cases from 1947 to 2016 in the literature and reported a reoperation rate of 48.1% (13 of 27).¹³⁾

The primary objective of surgical treatment for spinal GSD is stabilization. Surgical resection of the spinal GSD lesion with spinal column reconstruction is necessary for patients with spinal instability and deformity. Regarding surgical approach and reconstruction, few authors have reported successful spinal stabilization using only posterior pedicle screw instrumentation,⁹⁾ but most cases required anterior column reconstruction or three-column osteotomy.13,22,25,26)

The most critical factor in achieving successful stabilization in spinal GSD is the disease state. If the disease

is progressing, there is a higher chance of reconstruction failure and graft resorption (Fig. 3). Therefore, some authors recommend that spinal GSD surgery be performed only after disease progression has been controlled with systemic agents or radiotherapy.²⁵⁾ However, in spinal GSD patients with neurological deficits, early surgical intervention can be considered to yield a satisfactory neurological outcome.²²⁾

4. Local treatment

In addition to surgical treatment, percutaneous Fig. 3. (A) Whole-spine X-rays of a 12-year-old male patient with Gor- ham-Stout disease (GSD) in the lower thoracic region. (B) T1-weighted, contrast-enhanced sagittal magnetic resonance imaging shows extensive GSD involvement with acute angular kyphosis at the T10-11 level. (C) The patient underwent posterior decompression and stabilization using pedicle screw instrumentation. (D) At 5 years after surgery, instrumenta- tion had failed due to disease progression in the thoracic spine.

A

C D

B

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vertebroplasty can be effective in select cases of spinal GSD. Carbo in 2015²⁸⁾ and Liu in 2018¹⁴⁾ published case reports in which they performed vertebroplasty on patients with GSD in the lumbar spine. Patients from both studies did not experience any complications at 4 years and 2 years following vertebroplasty, respectively, and they did not require any additional surgery on spinal GSD. However, because percutaneous vertebroplasty in spinal GSD has a higher risk of cement leakage into the spinal canal when compared to other conditions, such as osteoporotic compression fractures, vertebroplasty should be performed with extreme caution in patients with spinal GSD (Fig. 4). Furthermore, the possibility of a revision surgery should always be explained to patients when vertebroplasty is considered for spinal GSD, which may progress after vertebroplasty.

A focal disease in the lumbar spine causing mechanical back pain, but without evident disease progression, can be a good candidate for percutaneous vertebroplasty. In contrast, extensive diseases and thoracic lesions are not considered good indications for vertebroplasty because of the higher risk of complications. Future studies should investigate proper indications and contraindications, adequate cement volume and viscosity, and management of lymphatic fluid in the vertebral body before injecting

bone cement (polymethyl acrylate).

Chylothorax is the most serious, often intractable, and fatal complication in GSD, especially in patients with thoracic spine involvement. Radiotherapy, pleurectomy, pleurodesis, thoracic duct ligation are available treatment options for chylothorax in GSD.^7,8) Among these options, many authors have reported successful treatment of chylothorax by thoracoscopy-assisted pleurodesis using various chemical agents.²⁹⁾ Spine surgeons treating GSD patients with the thoracic spine involvement should always be aware of this fatal complication and attempt to manage it with thoracic surgeons before considering surgical stabilization of the thoracic GSD.³⁰⁾

Conclusions

GSD is a rare, intractable disease that is characterized by massive osteolysis associated with the proliferation of lymphatic vessels. Although consensus is lacking regarding the appropriate treatment strategy for GSD because of its rarity, treatment for spinal GSD should be case-specific and involve multiple modalities, including systemic medications, radiotherapy, and surgical treatment.

Surgical treatment for spinal GSD should be considered after disease progression has been controlled with systemic Fig. 4. (A) Lumbar spine X-rays of a 63-year-old male patient showing compression fracture of the L1 vertebra due to Gorham-Stout disease (GSD) in- volvement. (B) T2-weighted and T1-weighted contrast-enhanced sagittal magnetic resonance imaging shows extensive GSD involvement in the lumbar spine with compression fracture at L1. (C) Percutaneous vertebroplasty was performed on L1 with no immediate postoperative complications. The pa- tient died 3 years following vertebroplasty due to refractory chylothorax.

A B C

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agents and radiotherapy, and maintenance treatment is also required postoperatively. Anterior column reconstruction is necessary for extensive diseases to reduce the high revision rate of spinal GSD surgeries.

Acknowledgment

The current review was presented at the 2020 Fall Case Discussion Symposium of the Korean Society of Spine Tumor.

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J Korean Soc Spine Surg. 2021 Jun;28(2):55-62

Review Article

Journal of Korean Society of Spine Surgery. www.krspine.org. pISSN 2093-4378 eISSN 2093-4386

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

척추를 침범한Gorham-Stout Disease의 치료

장삼열 • 김형민 • 장봉순 • 이춘기*

서울대학교 의과대학 정형외과학교실, *^{참조은병원 정형외과}

연구 계획: 종설

목적: 척추를 침범한 Gorham-Stout 병의 치료에 대한 최신 지견 소개

선행 연구문헌의 요약: Gorham-Stout 병은 림프 혈관의 증식으로 인해 진행하는 뼈 흡수를 특징으로 하는 매우 드문 질환이다. 여러 가지 치료 방법 들이 시도되었으나, 아직까지 어떠한 치료가 가장 효과적인가에 대해서는 아직 연구가 충분하지 않다.

대상 및 방법: 문헌 고찰 및 최신 연구의 소개

결과: Gorham-Stout 병의 치료에 사용될 수 있는 치료법에는 인터페론, 비스포스포네이트, 데노주맙, 시롤리무스 등의 약물 치료, 방사선 치료, 수술 적 치료와 척추성형술 등이 있다.

결론: Gorham-Stout 병은 질환의 상태(병변의 위치, 범위, 질병의 진행 여부 등)에 따라 치료가 달라지며, 여러 가지 방법들을 함께 활용하여 치료하 여야 한다.

색인 단어: Gorham-Stout 병, 데노주맙, 시롤리무스, 방사선치료, 척추성형술 약칭 제목: Gorham-Stout 병의 치료

접수일: 2021년 1월 6일 수정일: 2021년 1월 14일 게재확정일: 2021년 4월 27일

교신저자: 김형민

서울시 종로구 대학로 101 서울대학교병원 정형외과학교실

TEL: 02-2072-0357 FAX: 02-764-2718 E-mail: [email protected]