Case Presentation

The patient is 53. He had a normal childhood and had nothing wrong with his arm or shoulder until around age 30, when he developed a mass in his left neck.

Since then he has had development of innumerable masses in the left arm, the left chest wall, and the left shoulder. He has had by his estimation, 23 surgeries. The lesions would become larger and larger and he would call the doctor who would arrange to remove the most symptomatic ones. His last surgery was about 13 years ago.

The patient is generally healthy. He does not have hypertension, diabetes, heart disease, and he has never been a smoker. He has had chemotherapy, which he describes as given in pill form, type unknown, as well as 4,500 cGy radiation therapy to the left chest, shoulder, and arm.

Recently, he has had onset of lymphedema. This seems to have developed in the last three to six months. He has had development of large tortuous veins on the chest wall and the proximal portion of the arm. Recently his symptoms have been worsening rapidly. The pain in the arm is unrelenting. The arm has become a useless, painful burden to him. He would like to have the arm amputated.

The patient is retired from his job as an inspector at the electrical company in Connecticut. He has two daughters who are healthy. His wife passed away 7 years ago.

On the chest wall, there are dilated veins and erythema. However, there are no individual cutaneous tumors or hairy dark patches of skin. Clinical photos are shown at right.

The patient carries his arm in a makeshift sling. He has no useful motion of the arm. It is grossly swollen. There are multiple scars, as well as a thoracotomy scar. There are multiple palpable firm masses within the arm. The arm is exquisitely tender to the touch, and the dilated veins and areas of the chest wall adjacent to the arm are also quite tender.

Chest is clear to auscultation. The heart has a regular rate and rhythm, and S1 and S2 are normal. No extra heart sounds are heard. Abdomen is soft and nontender with no mass.

There is an MRI available. There are multiple nodular masses within the left upper extremity.

What is the diagnosis?

What could explain the recent worsening of the symptoms?

What work-up needs to be done?

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