Carcinosarcoma
Table I
Carcinosarcoma of the thyroid: summary of reported cases
| Saltykow13, 1905 | C-FS | 52/F | – | – |
| Chavannaz & Nadal14, 1910 | C-OS | 74/F | – | – |
| Zeckwer15, 1926 | C-FS | 61/M | None | 6 wk |
| Arons16, 1930 | C-FS | 56/M | Partial thyroidectomy | 2 mo |
| Nimpfer17, 1931 | C-OS | 62/F | – | – |
| Pinston18, 1958 | C-OS | 64/F | – | – |
| Cabanne19 et al., 1964 | C-OS | 60/F | – | – |
| Arean and Schildecker20, 1964 | C-FS | 61/F | Subtotal thyroidectomy | 1 yr |
| C-FS | 52/M | Subtotal thyroidectomy | 2 mo |
| Lira and Maranhao21, 1965 | C-OS | 68/M | Total thyroidectomy | 3 mo |
| Hedlinger22, 1969 | C-OS | – | – | – |
| C-OS | – | – | – |
| Heitz et al23, 1976 | C-OS | – | – | – |
| Donnell et al.24, 1987 | C-OS | 77/F | Subtotal thyroidectomy | 2 yr |
| Cooper and Barker25, 1989 | C-SA | 61/F | Subtotal thyroidectomy | > 5 mo |
| Hermes et al.26, 1992 | C-SA | – | – | – |
| Al Sobhi1, 1997 | C-SA | 80/F | Total thyroidectomy | 8 mo |
Carcinosarcoma of the thyroid gland is a rare but aggressive tumor. It arises from both the epithelial (carcinoma) and stromal (sarcoma) elements of the thyroid. Most cases occur in women who are older than years of age (
1) and usually present with a rapid growing thyroid mass. Some patients may have had a history of multimodular goiter. The exact pathogenesis is unknown. Fine Needle Aspiration Biopsy (FNAB) will demonstrate malignant cells: spindle and mesenchymal cells. Recently we have collected 17 patients from the literature (
Table 1). Treatment with total thyroidectomy and lymph node dissection is the only known effective treatment. Radiotherapy and chemotherapy are recommended because of the aggressive nature of this tumor. To date, no definite protocols are established for these patients.
Anaplastic Carcinoma
Anaplastic thyroid carcinoma is a rare and very aggressive tumor. It represents 5–10% of all thyroid carcinoma (2) and is more common in female than male patients (3.5/1). Patients usually present at about 60 years of age with a neck mass, dysphragia or change in voice or dyspnea. Multimodalities treatment are needed to treat these patients. Total thyroidectomy should be used whenever feasible followed by radiotherapy and chemotherapy. Some retrospective studies recently showed no difference in survival between patients who received surgery and those who did not. Most of these patients died from their disease. Chemotherapy is occasionally helpful but long term benefits are rare. Several modalities have been used. Some data suggest that paclitaxed may be helpful (3.6). High dose chemotherapy and hyperfractionation and radiotherapy after surgery improved survival in a recent retrospective study (5).
One randomized study compared doxorubicin with doxorubicin and cisplatin in 39 patients; a higher response to combined chemotherapy was demonstrated. Further studies and mulcentric trials should be initiated to find better ways to dignose an treat these patients.
Lymphoma of the Thyroid Gland
Prymary thyroid lymphoma is a rare disease. Most thyroid lymphomas are non-Hodgkin's of B-cell origin (7,8). There is a strong association between Hashimoto's Thyroiditis and lymphoma of the thyroid gland. The majority of these patients present with a neck mass; they are usually euthyroid.
Diagnosis and treatment
FNAB can usually distinguish between lymphoma and anaplastic carcinoma. Sometimes it is very difficult, however, to differentiate lymphoma from Hashimoto's thyroiditis. Immunohistochemical staining and flow cytometry, can help in the diagnosis of the lymphoma. After the diagnosis is established all patients should be staged. These tumors sre chemosensitive and radiosensitive and because they usually extend outside the thyroid gland, surgical resection alone is rarely curative. Thus radiotherapy chemotherapy is the prefered therapy. Different protocols have been used, including CHOP (Cyclophom, Sphamide, Adramycin, Vincristine, Prednisolone) (11,12).
Immature teratoma of Thyroid gland
This is a rare tumor. We have collected 14 cases in the English language literatures (9). Most teratomas in childhood are benign whereas teratomas in adults are usually malignant. These are aggressive tumors needing multimodality therapies. Total thyroidectomy followed by chemotherapy and radiotherapy is recommended. We have used 3 cycles of cisplatinum and VP-16 (9) for our patients. Most patients died of local recurrence or distant metastases.
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