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Lymphoma Involving the Mediastinum Challenges in Diagnosis

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Lymphoma Involving the Mediastinum Challenges in Diagnosis Lethal tracheal dissolution during treatment for thyroid lymphoma 1121 Figure 2 Portable chest radiograph after intubation to mention whether the tumour invaded the showing extensive trachea or larynx.4 In one report two patients subcutaneous emphysema demonstrated obstructive symptoms and bi- of the upper chest and opsy proven tracheal invasion by thyroid Thorax: first published as 10.1136/thx.50.10.1121 on 1 October 1995. Downloaded from neck. The cervical trachea is markedly distended lymphoma; both patients received chemo- (arrows). therapy with resolution ofobstruction and with- out tracheal perforation, and both were alive one and two years later without evidence of disease.5 Computed tomographic scanning is the re- commended method of assessment of extra- thyroid extension of lymphoma.' Early awareness of tracheal involvement by lymph- oma should alert the clinician to the remote possibility of tracheal dissolution during treat- ment of this extremely difficult clinical prob- lem. mon: hoarseness (9-67%), dysphagia (9-60%), 1 Randall J, Obeid ML, Blackledge GR. Haemorrhage and perforation of gastrointestinal neoplasms during chemo- and dyspnoea or stridor (9-35%).5 Although therapy. Ann R Coil Surg Engl 1986;68:286-9. the optimal treatment for thyroid lymphoma 2 Shaw JHF, Holden A, Sage M. Thyroid lymphoma. Br J Surg 1989;76:895-7. is uncertain, patients with stage IIE or more 3 Tupchong L, Hughes F, Harmer CL. Primary lymphoma of advanced disease should probably receive the thyroid: clinical features, prognostic factors, and results oftreatment. IntjRadiat OncolBiolPhys 1986;12:1813-21. chemotherapy in addition to local treatment 4 HamburgerJI, MillerJM, Kini SR. Lymphoma ofthe thyroid. (surgery and/or radiotherapy).6 Ann Intern Med 1983;99:685-93. 5 Van Ruiswyk J, Cunningham C, Cereletty J. Obstructive Perforation ofthe gastrointestinal tract at the manifestations ofthyroid lymphoma. Arch Intern Med 1989; site of disease in patients with gastrointestinal 149:1575-7. 6 Vigliotti A, Kong JS, Fuller LM, Velasquez WS. Thyroid lymphoma who are receiving chemotherapy has lymphomas stages IE and IIE: comparative results for been well described;' a similar perforation has radiotherapy only, combination chemotherapy only, and multimodality treatment. IntJ Radiat Oncol BiolPhys 1986; been reported in a patient with thyroid lymph- 12:1807-12. oma metastatic to the small bowel.7 7 McDermott EWM, Cassidy N, Heffeman SJ. Perforation through undiagnosed small bowel involvement in primary Lethal tracheal perforation during chemo- thyroid lymphoma during chemotherapy. Cancer 1992;69: therapy has not previously been described in a 572-3. 8 Takashima S, Morimoto S, Ikezoe J, Arisawa J, Hamada S, patient with primary thyroid lymphoma. Most Ikeda H, et al. Primary thyroid lymphoma: comparison of series of patients with thyroid lymphoma fail CT and US assessment. Radiology 1989;171:439-43. http://thorax.bmj.com/ Thorax 1995;50:1121-1123 found in the anterosuperior compartment. Commentary: Thymomas are the most common tumour in this region and lymphomas and carcinoids may lymphoma involving involve the thymus. Mediastinal germ cell tu- mours are located in the anterosuperior com- the mediastinum partment and thyroid tumours may involve on September 30, 2021 by guest. Protected copyright. the upper part of the superior mediastinum. challenges in diagnosis Secondary tumours, particularly bronchogenic carcinoma, should also always be considered. and management Thymomas are often suspected by their radio- logical appearance and they may have as- sociated systemic features, most notably myasthenia gravis. They are usually treated Jonathan A Ledermann by surgical extirpation which allows further detailed study of the pathology and avoids the Lymphomas are the seventh most common increased incidence of local recurrence seen if malignancy and the incidence of non-Hod- needle biopsy is performed before surgery. For gkin's lymphoma is increasing. Correct diag- other masses radiologically guided needle bi- nosis is important as they are treatable and, in opsy has become commonplace and has re- some cases, curable. Lymphomas can involve duced the need for operative biopsy. Fine any organ system and, as thoracic involvement needle aspiration is simple and may be sufficient is common, they often present to respiratory to diagnose a carcinoma. However, as Robinson physicians. The diagnosis is not always straight- et al point out, the diagnosis of lymphoma may forward and treatment is complex. The case be missed unless adequate tissue is removed. Department of reports in this issue of Thorax illustrate some This is required for study of the morphology Oncology, of the diagnostic and therapeutic problems as- of the tumour which provides prognostic in- University College sociated with this disease. formation and assists management. A com- London Medical Mediastinal tumours in adults are School, divided prehensive immunohistological examination London WIP 8BT fairly equally throughout the mediastinal com- should be performed in case misleading in- J A Ledermann partments. Approximately 40% of tumours are formation is obtained from one immuno- 122 Ledermann histochemical method. Some "anaplastic Cysts in the thymus such as those described carcinomas" turn out to be high grade non- by El-Sharkawi and Patel may be found when Hodgkin's lymphomas which are potentially this gland is the principal site of involvement curable.' Tumour-specific chromosomal ab- with Hodgkin's disease. Whether Hodgkin's Thorax: first published as 10.1136/thx.50.10.1121 on 1 October 1995. Downloaded from normalities have been identified in several types disease originates in the thymus in these cases of lymphoma, germ cell tumours, Ewing's sar- is not clear, but management is similar to me- coma, and peripheral neuroepithelioma. The diastinal Hodgkin's disease. diagnostic usefulness of this technique is likely A significant number of non-Hodgkin's to increase as further abnormalities are iden- lymphomas arise in extranodal sites. Their clin- tified. Raised serum tumour markers such as ical behaviour is related more to their biology oa-fetoprotein and human chorionic gonado- than to their anatomical location. Thyroid trophin may help to support a histological diag- lymphomas are rare and account for about nosis of a mediastinal germ cell tumour. Rarer 5% of thyroid malignancies. They are usually tumours such as the "atypical teratoma syn- associated with Hashimoto's thyroiditis and drome" should be considered if a rapidly grow- occur most commonly in women. Many are ing undifferentiated carcinoma is found in a low grade tumours similar to MALT lymphoma predominantly midline distribution. This found in the gastrointestinal tract.4 Others are tumour, which occurs particularly in young intermediate or high grade B cell tumours. men, often responds well to intensive cisplatin Although they are often confined to the thyroid based chemotherapy.2 and cervical lymph nodes at presentation, tu- Most cases ofHodgkin's disease that occur in mours with aggressive type histology may dis- the mediastinum are of the nodular sclerosing seminate widely.' T cell lymphomas of the type. The choice between primary chemo- thyroid are rare so that other sites of origin therapy and radiation is usually made on the should be considered, particularly as the patient basis of tumour stage and bulk of the disease. described by Melnyk et al had axillary lymph- A good clinical and radiological response to adenopathy and skin involvement. The tumour treatment occurs in most patients, but radio- in the patient they described had many char- logical abnormalities may persist with widening acteristics similar to those seen in peripheral T ofthe mediastinum and architectural distortion cell lymphomas ofthe angiocentric type. These of the lymph node areas. It is often impossible are aggressive tumours, associated with nec- to know whether there is persistent active rosis, and can involve the mediastinum and Hodgkin's disease. This creates a dilemma as lung. Tumours of the nasopharynx were for- consolidation treatment with radiotherapy may merly and appropriately called "lethal midline be required but it increases the chance of long granuloma". They behave as a locally de- term toxicity. An example of this problem is structive inflammatory tumour and evolve into presented in the report by Thomas et al. BCNU a generalised T cell lymphoma. They are known (carmustine) is one of a group of chemically to produce a coagulative necrosis6 and the case http://thorax.bmj.com/ unrelated drugs (others include bleomycin, described by Melnyk et al probably falls into busulfan, methotrexate, cyclophosphamide in this category. The destructive lesion they high doses, and mitomycin C) known for their described is dissimilar from the more widely damaging effects on lung. The probability of recognised "tumour lysis" syndrome, oc- pulmonary injury increases when radiotherapy casionally seen shortly after the start of chemo- is given to the lung, either before or after therapy for leukaemia and lymphomas. Rapid cytotoxic chemotherapy.3 Dry cough and dys- dissolution oftumour is usually associated with pnoea are the characteristic symptoms of acute a gross metabolic disturbance such as lactic pneumonitis due to cytotoxic drugs or ra- acidosis, hyperkalaemia, hyperphosphataemia, on September 30, 2021 by guest. Protected copyright. diation. Drug-related damage often causes and hyperuricaemic renal failure. These com- changes in the basal respiratory
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