Squamous Cell Carcinoma

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Primary squamous cell carcinoma is very rare, accounting for less than 1% of thyroid cancers, usually occurring in middle-aged or elderly patients [11]. The clinical behavior has been reported to resemble that of anaplastic carcinoma [12]. Prognosis is poor, with the majority of tumors advanced at presentation. The diagnosis should be made only once metastasis from other sites (such as the lung) and direct local invasion from tumors in adjacent structures (such as trachea or esophagus) are excluded. Squamous carcinoma must also be distin-

Figure 30.1 Royal Marsden Hospital policy for the management of primary thyroid lymphoma. CXR, chest radiograph; MALT, mucosa-associated lymphoid tissue; R-CHOP, rituximab, cyclophosphamide, hydroxydaunomycin (doxorubicin), vincristine (Oncovin), and prednisone; MPD, mid-plane dose, Ann Arbor Classification: Stage IE, disease in single extralymphatic site; Stage IIE, disease in extralymphatic site with its regional nodes; Stage III, disease in lymph node regions on both sides of the diaphragm affected; Stage IV, widespread disease, including multiple involvement at one or more extranodal (beyond the lymph node) sites, such as the bone marrow.

History and clinical examination Haematology, biochemistry, CXR, review histology CT neck, thorax, abdomen, pelvis Bone marrow aspirate and trephine

Stage IE or IIE

Surgery

Open biopsy (if not already done) Tracheostomy (only if unavoidable)

Stage III or IV

Surgery

Open biopsy (if not already done) Tracheostomy (only if unavoidable)

Chemotherapy

Partial response

consider additional chemotherapy

Complete response

Radiotherapy

35 Gy 20# 4 weeks involved field

Radiotherapy ^_

Simulate for anterior and undercouched Phase I to neck and mediastinum 2 Gy daily MPD: 40 Gy 20# 4 weeks no additional response

Follow-up clinic 3 monthly

Haematology and CXR

Complete remission

Recurrence

Chemotherapy guished from squamous metaplasia seen in papillary carcinoma, which is of no prognostic significance, and areas of squamous differentiation seen in anaplastic tumors [13]. Etiology remains uncertain but it may arise from embry-ological remnants such as the thyroglossal tract or from areas of squamous metaplasia within the gland. Alternatively it may develop directly from undifferentiated follicular cells, which differentiate into squamous cells during neoplastic transformation. Histological diagnosis requires identification of keratin or intercellular bridge structures. Immunohistochemistry is helpful, with negative staining for thyroglobulin and calcitonin.

Squamous cell carcinoma of the thyroid follows an aggressive clinical course [13]. We have reported 16 patients of whom 12 had locoregional disease only at presentation and 4 had distant metastases [14]. Eight underwent surgery (5 complete and 3 incomplete resection) with 4 given postoperative radiotherapy. Radiotherapy alone was used in 6 patients unsuitable for surgery. Median survival was only 16

months, although there were 3 long-term survivors treated with complete resection followed by radiotherapy. Patients treated with surgery alone all developed local recurrence. Sarda et al. reported 8 patients, none of whom were amenable to radical surgery [15]; none displayed any significant response to either radiotherapy or chemotherapy. Similarly, Shimaoka et al. described 5 patients who failed to respond to chemotherapy or radiotherapy and all died of local disease [13].

Simpson and Carruthers suggested that early complete macroscopic excision followed by external beam radiotherapy can offer the best chance of cure and local control, having described two patients displaying long-term survival [16]. Our series supports this recommendation, with three patients treated in this fashion remaining disease-free, in contrast to two who underwent complete macroscopic resection without irradiation developing local recurrence.

We recommend radiotherapy after total thyroidectomy to a dose of at least 60 Gy in 30 fractions over 6 weeks [17]. The initial target volume should include potential areas of lymph node spread up to a dose of 46 Gy (spinal cord tolerance) with a further 14Gy using 3D conformal planning. Our data support reports that radiotherapy alone or following incomplete resection results in poor local control. In seven patients treated with radiotherapy following biopsy alone, the median survival was only 6 months. However, high dose palliative irradiation for inoperable tumors may be beneficial. Consistent with other reports, chemotherapy is of little value and radioiodine is without benefit as these tumors are non-iodine avid.

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