Chest wall swelling: unusual presentation of an aggressive mediastinal tumour

Abstract

The majority of patients with primary mediastinal lymphoma are symptomatic at the time of diagnosis and commonly have fever, weight loss and/or night sweats. Symptoms due to compression of adjacent mediastinal structures are infrequent, but may include pain, dyspnoea, stridor, or superior vena cava syndrome. Local infiltration into the chest wall, pleura and pericardium is not uncommon.

In the present report, two interesting cases of chest wall swellings that in fact were extensions of primary mediastinal lymphoma are given. Histopathology of the tumour was large B cell lymphoma (CD20+). The first case was in a 23-year-old woman, with dramatic onset but a good outcome. The second was in a 34-year-old Pakistani woman, with insidious onset and poor outcome due to extent and invasion by the tumour. Interesting CT images are presented showing chest wall and left supraclavicular swelling.

Background

All left supraclavicular swellings need investigation for lesions other than metastases from the stomach, testicles, ovaries, pancreas and so on. Chest wall swellings may not always arise from constituents of the chest wall but may represent an extension of the mass inside. Acute presentation of aggressive lymphomas is not unusual, and tissue biopsy is essential to establish the diagnosis and plan treatment. Surgery has no role in the treatment of mediastinal lymphoma, and symptoms may not be present at presentation.

Case presentations

Case 1

A 23-year-old woman presented with a 2-week history of swellings on the chest wall and left side of the neck. The patient gave a history of a mild, boring pain in the left parasternal region but no experience of fever, weight loss or night sweats and she denied any personal or family history of tuberculosis. The pain was relieved by analgaesics such as paracetamol and ibuprofen initially, but became continuous and was only relieved by tromadol. Examination revealed an average woman with a firm to hard mass of 5×5 cm on the anterior chest wall adjacent to the sternum, slightly painful on pressure (fig 1), and a left supraclavicular lymph node 3.5×4 cm, firm, non-tender and mobile (fig 1). Complete blood counts were normal, and the erythrocyte sedimentation rate was 115 mm/h. Serum transaminases were normal as was serum lactic dehydrogenase and uric acid. The possibility of a tumour arising from chest wall structures was considered with the following differential diagnoses (table 1).

Figure 1.

Anterior chest wall swelling and left supraclavicular swelling.

Table 1.

Chest wall lesions

Lesion type	Name/description
Benign	Osteochondroma
	Chondroma
	Langerhan cell histiocytosis
Malignant	Chondrosarcoma
	Ewing sarcoma family of tumours (EFT): Askin tumour of the chest wall and primitive neuroectodermal tumour (PNET)
	Osteosarcoma
	Solitary plasmacytoma

In view of the left supraclavicular lymph node, other possibilities were discussed (table 2).

Table 2.

Differential diagnoses of left supraclavicular masses

Lesion type	Name/description
Benign lesions	Rare, such as tubercular lymph node or cold abscess lesions
Malignant (“Virchow node”)	Metastases from the stomach, gallbladder, pancreas, kidneys, testicles, ovaries, or prostate
	Mediastinal metastases (right supraclavicular fossa)
	Metastases from mediastinum and lung

However, x ray of the chest showed a large mediastinal mass, which on CT scanning was revealed to be a large mass extending beyond the anterior chest wall and sternum (fig 2), encasing the adjacent vessels. Sections at the level of the neck revealed left supraclavicular swelling consistent with a lymph node. Fine needle aspiration of the masses and a mediastinascopic biopsy were inconclusive. A CT scan of the abdomen revealed hepatosplenomegaly, but bone marrow aspiration was negative for any infiltration. An incisional biopsy of the chest wall mass proved it to be a diffuse large cell B cell lymphoma (CD 20+) (fig 3). The patient was diagnosed as having a primary mediastinal lymphoma of the aggressive non-Hodgkin’s type. The patient was given cyclophosphamide, vincristine, doxorubicin, prednisolone and rituximab (CHOP-R) chemotherapy and is so far doing well.

Figure 2.

CT scan of the chest and mediastinum showing tumour extension beyond the sternum and contiguous with chest wall swelling.

Figure 3.

Biopsy of mass showing large cell lymphoma (H&E stain).

Case 2

Case 2 was a 34-year-old Pakistani woman, who presented with non-productive cough, fever and weight loss of 6-months duration. Examination revealed an average build woman with a tender subcutaneous mass anterior to the sternum and cervical and axillary lymphadenopathy. An x ray of the chest showed mediastinal widening; complete blood counts were normal, and the erythrocyte sedimentation rate was 102 mm/h (first hour). CT of the chest revealed a huge lobulated mass with areas of necrosis, eroding the sternum and extending subcutaneously (fig 4). There was left-sided pleural effusion but no hepatosplenomegaly and no bone marrow involvement. A lymph node biopsy revealed a large cell lymphoma (CD 20+). The patient was subjected to radiotherapy to decrease the tumour bulk, but unfortunately died of a massive bout of bleeding from the upper respiratory tract due to invasion of the trachea by the tumour.

Figure 4.

CT scan of the chest of patient 2 showing a mediastinal mass eroding the sternum and encasing mediastinal structures, with areas of necrosis.

Investigations

• CT scans.

• Biopsies from masses and lymph nodes.

• Bone marrow aspiration biopsies.

Differential diagnosis

See table 2.

Treatment

Patient 1 was given CHOP-R chemotherapy and is so far doing well. Patient 2 was treated with radiotherapy

Outcome and follow-up

Patient 1 is doing well after 1 year of treatment. Patient 2 unfortunately died of a massive bleed.

Discussion

The anterior mediastinal compartment is anterior to the pericardium and includes lymphatic tissue, the thymus, the extrapericardial aorta and its branches, and the great veins. Masses in the anterior compartment are more likely to be malignant than those found in the other mediastinal compartments. Lesions most commonly found in the anterior mediastinum are thymomas, germ cell tumours, lymphomas, intrathoracic thyroid tissue lesions and parathyroid lesions.¹

Although patients with disseminated systemic lymphoma often have involvement of the mediastinum, 5% to 10% of patients with lymphoma present with primary mediastinal lesions. Primary mediastinal lymphoma accounts for 10% to 20% of primary mediastinal masses and is the second most common primary anterior mediastinal mass in adults.² Most mediastinal lymphomas are seen in the anterosuperior mediastinum, with the remainder being found in the middle mediastinum.

Primary mediastinal Hodgkin’s lymphoma occurs predominantly in the third and fourth decades, and is usually of the nodular sclerosing variety.³ Non-Hodgkin’s lymphoma is found in all age groups but is more common in women in the third and fourth decades.²

The majority of patients with primary mediastinal lymphoma are symptomatic at the time of diagnosis and commonly experience fever, weight loss and/or night sweats. Symptoms due to compression of adjacent mediastinal structures are infrequent, but may include pain, dyspnoea, stridor, or superior vena cava syndrome. Local infiltration into the chest wall, pleura and pericardium is not uncommon.³ Our patient presented with swelling of the chest and a swelling in the neck in addition to chest pain. The diagnosis is initially suggested by radiographic studies demonstrating a lobulated mass in the anterosuperior mediastinum. Tissue confirmation of the diagnosis is mandatory, and may be accomplished by excision of an enlarged cervical or supraclavicular lymph node (if present), mediastinoscopy, mediastinotomy or thoracotomy. Fine needle aspiration biopsy usually does not provide adequate tissue to establish the diagnosis.

Treatment of mediastinal Hodgkin’s lymphoma consists of chemotherapy and/or radiotherapy. The 5-year survival rate has improved dramatically over the past 20 years, and is currently approximately 75%.

Mediastinal lymphoma is usually a locally invasive tumour where surgical resection can play no role. In rare cases where a tumour is identified that is readily resectable, complete excision with adjuvant therapy may be indicated because favourable results with this approach have been previously reported with lymphomas at other extranodal sites.

In contrast to the improved results in patients with mediastinal Hodgkin’s lymphoma, mediastinal non-Hodgkin’s lymphoma is still associated with a poor prognosis. If treatment is followed as per international prognostic index (IPI) the outcome is variable, and the lower the index at the start of treatment the better the 5-year disease free survival rate; the higher the IPI, the worse the 5-year disease free survival rate.⁴

Learning points

• All left supraclavicular swellings need investigation for lesions other than metastases from the stomach, testicles, ovaries, pancreas and so on.

• Chest wall swelling may not always arise from the constituents of chest wall, but may represent an extension of the mass inside.

• Acute presentation of aggressive lymphomas is not unusual, though symptoms may not be present at presentation.

• Tissue biopsy is a must to establish the diagnosis and plan treatment.

• Surgery has no role in treatment of mediastinal lymphoma.