Huge "chest muscles", actually hidden tumor risk?

Case introduction

Uncle Wang (pseudonym) is a fitness expert, every day at 3 p.m. will not move to the downstairs sports field for fitness, in the past 3 months, Uncle Wang exercise effect is extraordinary, the left pectoral muscles are getting bigger and bigger day by day. Could it be that exercise has an effect? Uncle Wang was a little flustered in his heart, and he always felt that something was wrong. The daughter noticed Uncle Wang's worries and worried about whether there was a physical problem, so she took him to Shanghai Meizhongjiahe Medical Imaging Diagnostic Center for PET/CT examination.

It doesn't matter, it turned out to be ligament-like fibromatosis!

Imagery

PET/CT images: a large area of abnormally thickened soft tissue mass (mainly composed of swollen pectoralis major muscles and pectoralis minor muscles, some of which are not clearly demarcated from the thickened skin), the density is uneven, reaching up to the left shoulder peak, the boundary between the adjacent deltoid muscles is not clear, the median line is crossed inward, close to the sternal stalk and body, the invasion of the right pectoralis major muscle causes the medial part of it to swell, the skin thickens in the corresponding area, the subcutaneous fat gap is blurred, the intercostal muscle is invaded, and protrudes into the left chest cavity and the anterior upper mediastinum, and the lesion density is uneven, FDG metabolism increased, with a SUVmax value of 4.5. Diagnostic considerations: low-grade malignancy and invasion of surrounding tissues.

After the PET/CT evaluation, the doctor successfully removed this huge "pectoral muscle" and treated the surrounding violated tissue, and also formulated a follow-up rehabilitation plan for Uncle Wang.

Postoperative pathology suggests a rare type of low-grade malignancy: ligament-like fibromatosis.

So what is ligament-like fibromatosis? Let's get to know this rare disease.

Ligament-like fibromatosis

Demoid-type fibromatosis, also known as aggressive fibromatosis or desmoid tumor, is a fibroblast that occurs in fascia, tendon membranes, or deep tissues and is formed by the overgrowth of fibroblasts and myofibroblasts, often growing invasively into adjacent muscle or adipose tissue. Sometimes the tumor can also invade adjacent important structures or parenchymal organs, and it is easy to recur after resection, so it is clinically called low-grade malignant or intermediate tumor.

Causes of ligament-like fibromatosis

Ligament-like fibromas are sporadic, partially familial, no racial differences, can occur at any age, the highest incidence is between 20 and 40 years old, and the incidence in women is 1 to 3 times that of men. The incidence ranges from 2 to 5 parts per million, accounting for 0.03% of systemic solid tumors and 3.6% of fibrous tissue solid tumors, which is rare. The cause of the disease is unclear and may be a causative process of multifactorial interaction. Patients with invasive fibromas of the abdominal wall often have a clear local trauma (eg, history of pregnancy, surgery) or radiation exposure, suggesting that physical factors such as trauma and radiation injury may be a precipitating mechanism for the formation of the disease. In addition, studies have confirmed that endocrine hormones such as estrogen and prostaglandins can promote the growth and development of some ligament-like fibromas.

Clinical features

Clinically, it is mainly divided into three categories according to the anatomical site of its occurrence: external abdominal ligament-like fibromatosis, abdominal wall and pelvic ligament-like fibromatosis, and intra-abdominal ligament-like fibromatosis. Extra-abdominal lesions occur mostly in young men, mostly on the shoulders, chest wall, back, thigh muscles, or extremities. Lesions located deep in the depths often form hard, unambiguous lumps with occult growth and rarely cause pain. Some patients have radiating pain or tingling, numbness, sluggishness, and weakened mobility due to tumor infiltration or enveloping nearby nerves, which can cause paraplegia, paraplegia, and even fatality in severe cases.

Uncle Wang's case is external ligament-like fibromatosis in the abdomen, which has the characteristics of deep occult growth, unclear boundaries of the mass, and violation of surrounding tissues.

Imaging presentation

Imaging features of ligament-like fibromatosis resemble normal connective tissue or sarcoma. Less common in ct low-density areas, may indicate mucus components, are more common in chest wall disorders, may have different CT values, are similar to or slightly higher than skeletal muscle, and may reflect collagen and mucus components, respectively. Enhancement can be significantly strengthened, possibly the abundant capillary network in these tumors. MRI can be used for initial diagnosis, local surgical staging, and follow-up. Its superior soft-tissue contrast allows for accurate assessment of tumor boundaries and their relationship to adjacent structures, including muscles, nerves, blood vessels, and bones. The T2-weighted signaling of the mass reflects the number and distribution of histological components such as myofibroblasts, extracellular collagen, and mucus-like matrix, which may be associated with clinical behavior, suggesting consideration of the diagnosis of ligament-like fibromatosis. T1WI is mostly equal intensity or slightly high intensity signal, T2WI is uneven slightly high intensity signal, which has preoperative diagnostic value. In about 86% of cases, T2 weighting shows bands of low signal intensity (corresponding to histologically collagen fiber bands).

Unlike soft-tissue sarcoma, ligament-like fibromatosis, despite its lack of metastatic potential, often recurs locally after resection and is classified as an intermediate/locally invasive tumor by the World Health Organization (WHO). Imaging plays an increasingly important role in postoperative follow-up monitoring of these patients, and the first MRI evaluation should be performed 4-8 weeks after surgical treatment to avoid missing out on rapidly progressive disease, especially for lesions that are not easily accessible or are located in key areas. If MRI suggests stability, follow-up every 3 months in the 1st year thereafter, every 6 months in the 2nd to 5th year, and annually after the 5th year.

Any soft tissue mass with the following clinical features require diagnosis at a specialist hospital: size > 5 cm; the mass is deep to deep fascia; the mass is enlarged; and pain or recurrence of a previously removed site is indicated. Once ligament-like fibromatosis is confirmed, monitoring can be performed using a simplified MRI protocol.

Although PET/CT is not a conventional means of assessing or monitoring ligament-like fibromatosis, it has been proposed as a potential prognostic marker for distinguishing responders from non-responders in patients treated with medication. PET/CT can also be a good assessment of the extent of violation of surrounding tissues.

treat

Surgical resection is the most effective means of treating this disease, and the total cure rate of surgery is nearly 90%. Surgical margins are clearly related to postoperative recurrence, with few recurrences of negative margins and high recurrences of positive margins, so negative margins are advocated as the criterion for radical resection. For locally recurrent tumors, resection is mostly advocated.

The therapeutic effect of radiotherapy for ligament-like fibromatosis has been recognized, and is mainly used in patients who cannot be surgically removed (such as large nerves, blood vessels, or vital organs around tumors), difficult to completely resect, positive for the resection margin or adjacent to the tumor, and patients who have repeated relapses and lost the opportunity to re-operate.

Drug treatment of ligament-like fibromatosis mainly includes nonsteroidal anti-inflammatory drugs, anti-estrogen drugs, and cytotoxic chemotherapy drugs. NSAIDs and anti-estrogens can be used preoperatively or postoperatively. Cytotoxic chemotherapy is mainly used for tumors that cannot be resected or radiotherapy, and in patients who have failed non-cytotoxic drug therapy, and have a certain effect on deep intra-abdominal fibromatosis.

Through the above introduction, the discovery of ligament-like fibromatosis is not terrible, as long as it is detected early, surgically removed, the margin tissue is negative and regular imaging review, it is just a small episode of life.

Pay attention to U.S.-China Jiahe to protect your health.

Thanks to the reviewers for this issue

Shou Yi

Deputy Chief Physician Master Supervisor

Shanghai MeizhongJiahe Medical Imaging Diagnostic Center

Director of the Department of Nuclear Medicine

Ph.D. in Imaging Medicine and Nuclear Medicine. He has worked in Renji Hospital affiliated to Jiaotong University School of Medicine and Dongfang Hospital affiliated to Tongji University for more than 20 years, and has been engaged in imaging medicine and nuclear medicine imaging diagnosis for a long time, and has rich experience in PET/MR, PET/CT and SPECT/CT fusion imaging diagnosis. He has presided over 2 projects of Shanghai Municipal Science and Technology Commission and Shanghai Municipal Health and Family Planning Commission, participated in a number of other national and provincial and ministerial projects, published more than 40 papers, participated in the compilation of 5 books, and is currently a member of the PET Group of the Nuclear Medicine Branch of the Chinese Medical Association, a young member of the Nuclear Medicine Physician Branch of the Chinese Medical Doctor Association, a young member of the Nuclear Medicine Branch of the Shanghai Medical Association and a member of the PET Science Group, a member of the Nuclear Medicine Committee and the Leader of the Youth Studies Group of the Shanghai Nuclear Society, and a member of the Nuclear Medicine Branch of the Shanghai Association of Integrative Traditional chinese and Western Medicine.

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bibliography

1.Xiao Y, He J, Gong C, Liu L, Huang S. Desmoid-type fibromatosis of the chest wall: a case report. Ann Transl Med. 2020;8( 20):1322. doi:10.21037/atm-20-5237.

2. Garcia-Ortega DY, Martín-Tellez KS, Cuellar-Hubbe M, et al. Desmoid-Type Fibromatosis. Cancers (Basel). 2020;12(7):1851. Published 2020 Jul 9. doi:10.3390/cancers12071851

3.Goldblum J，Fletcher JA. Desmoid-type fibromatosis. In:Fletche CD，Unni KK，Mertens F，eds.World Health Organization classification of tumors:pathology and genetics of tumors of soft tissue and bone. Lyon:IARC Press 2002;83-84.

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