"Disappearing beautiful legs" 1, 2... More than 10 marble-sized lumps have caused many hospitals to be troubled

Ling Hua (pseudonym of the patient) pointed to the bean-sized lump on her left calf and asked the doctor with frustration but full of expectation.

She originally had a pair of slender "comic legs", but now they have swollen into "elephant legs". What makes her even more worried is that this "elephant leg" is inexplicably covered with lumps of various sizes, there are more than 10 of them.

This is not the first time Ling Hua has been hospitalized. As early as 2018 and 2019, Ling Hua underwent two operations for " endometrial adenocarcinoma " and " right colon adenocarcinoma ". She was secretly worried that it would be some other cancer this time?

After a careful physical examination and lower limb MRI, radionuclide lymphoscintigraphy, lower limb vascular ultrasound and other examinations, the doctor determined that the cause of Linghua's swollen legs was basically " lymphedema " on the basis of excluding diseases such as tumor recurrence, tuberculosis, and filariasis. To be more precise, it was " secondary lymphedema of both lower limbs ", which is a pathological condition characterized by accumulation of fluid in the superficial soft tissue of the limbs due to obstruction of lymph return due to various reasons, followed by hyperplasia of fibrous connective tissue, fat sclerosis, thickening of fascia and thickening of the entire affected limb.

At this moment, Ling Hua understood that perhaps it was the multiple factors of cancer that caused her "comic legs" to disappear. The swelling that replaced them was not abnormal, but the blocked lymph fluid in her body was causing the problem.

"Doctor, besides swelling, will this lymph node obstruction have any other adverse effects on me? Are these lumps on my legs related to it?" Ling Hua was eager to know.

The doctor further explained to her: " The lymphatic system is a relatively independent second circulatory system of the human body. It has important physiological functions such as maintaining tissue fluid balance, immune defense, and lipid metabolism. When the lymphatic system is damaged, some tissue fluid will be blocked from returning and retained in the tissue, causing edema in local tissues and organs, just like the swelling in the leg now. "

"Of course, in addition to causing tissue swelling, lymphedema can also cause pathological changes such as chronic inflammation and fibrosis of the swollen tissue. These lumps may be related to this, and this cannot be ruled out at present."

The doctor then added.

"Then what should I do? Doctor, do you have any suggestions? I don't want to be tied up by these thick legs and these big and small lumps like this..."

As she spoke, Ling Hua began to choke up.

Yes, 10 years ago, at the most beautiful age, she unfortunately suffered from hyperthyroidism and underwent several nuclear medicine treatments. Although the disease was effectively controlled, she still needed to take medication for life. In the past two years, she has almost exhausted all her courage in the fight against cancer. She has been brave enough to face surgery again and again. She always believes in the power of life, that she is still young, and that there is still a long way to go in the future...

However, fate did not seem to relax its tests on her. It took away her important organs and continued to devour her health and her desire and pursuit of beauty.

Seeing this, the doctor comforted her and said, "You don't have to have too much psychological pressure. Emotions will also affect your recovery. According to the results of auxiliary examinations, the preliminary diagnosis of multiple lumps in the lower limbs is that they may be benign."

Linghua did not understand the concept of probability in medicine, but in her opinion, the word "benign" was far more important than "possible".

She excitedly said to the doctor: "It's good if it's benign, it's good if it's benign! Thank you!" However, the doctor thought for a moment and added the second half of the sentence: "But I suggest you still do a biopsy to take some tissue from the many lumps for pathological analysis and combined with pathological diagnosis to clarify the pathological condition of the leg skin tumor and whether it is benign or malignant."

Linghua seemed to understand that the pathological analysis of the lump on her leg was the key to determining whether it was benign or malignant . But she didn't know that the doctor had left a half sentence unsaid but remained in her mind at that moment - limbs with lymphedema have a high tendency to be infected by bacteria, fungi and viruses, and have a higher risk of developing malignant tumors and metastasis .

In previous clinical diagnosis and treatment, a certain proportion of patients with malignant tumors had lower limb lymphedema as their first symptom. The onset of these patients was insidious, and this type of edema was often very similar to lower limb edema caused by vascular, hepatic, renal, cardiac and other diseases, making it difficult to distinguish.

Doctors once found in a literature study that 33 patients were diagnosed with primary lower limb lymphedema caused by non-malignant factors before hospitalization. Further examination after admission revealed that all of them were caused by infiltration and metastasis of malignant tumors.

Therefore, when diagnosing lower limb lymphedema, one must first consider whether it is caused by the recurrence and metastasis of malignant tumors. This is also the real reason why he recommended Linghua to undergo a biopsy. Frankly speaking, the doctor did not want Linghua to suffer from cancer again, or any other borderline cancer.

According to the doctor's treatment plan, Ling Hua underwent a leg skin biopsy as scheduled. A small piece of the skin lump on her left calf was removed, measuring 3.5 cm×1.5 cm×0.9 cm. The biopsy went smoothly, but the days of waiting for the pathology results were extremely torturous for her.

Of course, her attending physician was also suffering as she did.

Although the doctor found the cause of her "elephant legs", he still could not give a clear reason for the lumps of varying sizes on her legs. She felt like there was a little rabbit in her heart, rolling up and down.

If Linghua's nodules are really malignant, then it will be a tough battle...

A few days later, the pathology results came out, indicating:

Gross examination: a piece of skin and subcutaneous tissue, 3.5 cm × 1.5 cm × 0.9 cm in size, skin area of ​​(3.6 × 1.1) cm2, cut surface showed a nodule of 1.5 cm × 1 cm × 0.7 cm in size, gray powdery, solid, and unclear boundaries;

Microscopic examination: hyperkeratosis of squamous epithelium, myxoid degeneration of dermis, scattered astral cells, and no cell atypia; combined with clinical and immunohistochemical results, the diagnosis was fibromyxoid tumor (intermediate type), tending to be superficial CD34-positive fibroblastic tumor with myxoid degeneration. Immunohistochemical results showed P53 (5%+), Ki-67 (index 5%), SOX10 (-), S-100 (-), SMA (-), D2-40 (-), CD34 (+), CD31 (vascular +).

The pathological diagnosis made everyone fall into deeper confusion. The doctors disagreed on whether Linghua had a malignant tumor or a borderline tumor, and they were unable to give Linghua an accurate answer immediately...

Since the pathological section of the leg lump was formed in March 2021, Linghua did not learn the true identity of the leg lump until nearly 6 months later.

During this period, Linghua's pathological slide went through many hospitals. If it could tell its own story, it might record its magical journey like this:

April 12, 2021

I went to Hunan Cancer Hospital today, and the doctor from the pathology department told me that I have "superficial angiomyxoma and superficial acrofacial fibromyxoma". I mean, this name is too long...

April 27, 2021

Today I met with a doctor from the Pathology Department of the Cancer Hospital of the Chinese Academy of Medical Sciences. He thought that I met the criteria for "CD34-positive fibroblastoma with abundant mucinous lesions". If I told Linghua this, she would definitely be confused by this professional term.

May 21, 2021

The dermatologist at Peking Union Medical College Hospital suspected that I had "pretibial myxedema" and recommended that I apply halometasone cream to my "base camp" and wrap me in plastic wrap... It seems that this time the diagnosis and treatment are serious.

August 17, 2021

I didn’t expect that no one had guessed who I was yet. Today, I came to the Pathology Department of Beijing Jishuitan Hospital again. Like a previous analysis, they also thought that I had “superficial angiomyxoma.”

August 19, 2021

The Department of Pathology, Peking University Health Science Center/Department of Pathology, Peking University Third Hospital, consulted and concluded that I might have "secondary changes of lymphatic stasis, with myxedema not ruled out."

Finally, after going back and forth to so many hospitals, we finally agreed that I had " left anterior tibial myxedema ", also known as thyrotoxic mucinosis , which refers to the thickening and hardening of the skin on the front of the lower leg (the front of the tibia) of the patient, with an uneven surface. The skin lesions appear as plaque-like nodules of varying sizes, which are round, oval or irregular in shape. Because the lesions are mostly localized, they are also called " localized myxedema ." Linghua finally understood what the lump that had troubled her for more than a year was, and the "pathological diary" of the "mysterious tumor" could come to an end.

Having escaped a malignant tumor and uncovered the mystery of "elephant legs" and leg tumors, Ling Hua finally felt half relieved. The remaining half was worrying about whether this disease could be cured.

In fact, the answer to this question has long been found in the "Pathology Diary". In May 2021, the Department of Dermatology of Peking Union Medical College Hospital recommended applying topical halometasone cream to the affected limb and wrapping it with plastic wrap . In June, Linghua began to try this treatment plan, and soon the local hormone treatment had a significant effect. After 4 days of medication, the lump was gradually reduced during follow-up.

April 9, 2022 is a happy day for Linghua. On this day, she dressed up carefully and came to the hospital again. When everyone admired the smooth changes in her leg skin, she showed a long-lost and bright smile. More importantly, this time the doctor told her with certainty that "there are no signs of malignant tumors in the whole body."

The doctor sighed, "In fact, your leg is secondary lymphedema. Anterior tibial myxedema is even rarer and is particularly easy to misdiagnose and mistreat. We can clearly see the symptoms of your legs, and you really have half the credit! Do you regret going to so many hospitals?"

Ling Hua smiled but didn't answer. Walking out of the hospital with ease was already her answer.

▌This article is based on the column of Difficult and Rare Diseases in the 4th issue of "Journal of Peking Union Medical College" in 2023, "A Case of Secondary Lymphedema of Both Lower Limbs Combined with Myxedema of the Left Anterior Tibia"

Original author: Hao Kun, Sun Yuguang, Li Bin, Xin Jianfeng, Zhang Li, Yu Chunkai, Wang Rengui, Shen Wenbin

Text rewriting: Wang Yan, Liu Yang

【 References 】

[1] Hao Kun, Sun Yuguang, Li Bin, Xin Jianfeng, Zhang Li, Yu Chunkai, Wang Rengui, Shen Wenbin. A case of secondary lymphedema of both lower limbs combined with left anterior tibial myxedema[J]. Journal of Peking Union Medical College, 2023, 14(4): 855-858.

[2] Hao Kun, Xin Jianfeng, Sun Yuguang, et al. Clinical analysis of malignant tumors with lower limb lymphedema as the first manifestation[J]. Chinese Medical Journal, 2022, 102: 584-587.

[3] Ruocco V, Brunetti G, Puca RV, et al. The immunocompromised district: a unifying concept for
lymphoedematous, herpes‑infected and otherwise damaged sites[J]. J Eur Acad Dermatol Venereol, 2009, 23(12):1364‑1373. DOI: 10.1111/j.1468‑3083.2009.03345.x.

Editor: Liu Yang and Zhao Na

Proofreading: Li Na, Li Yule, Dong Zhe, Li Huiwen

Producer: Wu Wenming

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