Ten Best Readings
Ten Best Readings On Endocrine Malignancies
Peter J. Fabri, MD
University of South Florida College of Medicine, Tampa, Fla
The ten best recent articles in the medical literature relating to endocrine
malignancies are reviewed here.
Crucitti F, Bellantone R, Ferrante A, et al. The Italian Registry for Adrenal
Cortical Carcinoma: analysis of a multi-institutional series of 129 patients. The ACC
Italian Registry Study Group. Surgery. 1996;119:161-170.
The poor prognosis of adrenal cortical carcinoma may be improved by early diagnosis and
complete resection. Radical surgery is the sole effective therapy, particularly in early
stages. Surgical treatment of recurrence seems to improve survival and should be attempted
systematically.
DeLellis RA. Multiple endocrine neoplasia syndromes revisited: clinical, morphologic,
and molecular features. Lab Invest. 1995;72:494-505.
The author presents a well-written review that emphasizes the biology of the multiple
endocrine neoplasia syndromes as the basis for good care.
Eng C. The ret proto-oncogene in multiple endocrine neoplasia type 2 and
Hirschsprung's disease. N Engl J Med. 1996;335:943-951.
Mutations in the ret proto-oncogene are associated with some familial forms of
Hirschsprung's disease. In the case of multiple endocrine neoplasia type 2, the ret
mutations are activating (ie, they enhance the function of the encoded protein), whereas
in Hirschsprung's disease, the mutations are inactivating and lead to loss of function. In
rare families, Hirschsprung's disease and multiple endocrine neoplasia type 2 cosegregate.
Marsh DJ, Learoyd DL, Robinson BG. Medullary thyroid carcinoma: recent advances and
management update. Thyroid. 1995;5:407-424.
There is general agreement that the primary operation for medullary carcinomas of the
thyroid should include total thyroidectomy and central neck lymph node clearance. The role
of microdissection for recurrent disease awaits longitudinal evaluation. External
radiotherapy, radionuclide therapy, and chemotherapy may have roles in palliation but have
not definitively shown a curative value.
McNicholas MM, Lee MJ, Mayo-Smith WW, et al. An imaging algorithm for the
differential diagnosis of adrenal adenomas and metastases. AJR Am J Roentgenol.
1995;165:1453-1459.
An algorithm was developed for diagnosis of adrenal lesions that uses the density reading
on noncontrast computed tomography scan as the first step, with chemical-shift magnetic
resonance imaging for CT-indeterminate lesions. The algorithm is cost-effective and
reduces the number of biopsies required without reducing the sensitivity of detecting
malignant lesions.
Pia A, Berruti A, Terzolo M, et al. Feasibility of the association of mitotane with
etoposide, adriamycin and cisplatin combination chemotherapy in advanced adrenocortical
cancer patients: report on seven cases. Ann Oncol. 1995;6:509-510.
This small pilot study underscores the need for more effective systemic approaches for the
treatment of advanced adrenocortical cancer.
Sloan DA, Schwartz RW, Kenady DE. Surgical therapy for endocrine tumors of abdominal
origin. Curr Opin Oncol. 1993;5:100-109.
Surgery continues to be the primary treatment of endocrine neoplasia. The surgical
management of endocrine tumors of the pancreas, the adrenal glands, and the
gastrointestinal tract (specifically carcinoid tumors) are reviewed.
Eng C, Clayton D, Schuffenecker I, et al. The relationship between specific ret
proto-oncogene mutations and disease phenotype in multiple endocrine neoplasia type 2. JAMA.
1996;276:1575-1579.
This consortium analysis suggests that genotype-phenotype correlations do exist and, if
made reliably absolute, could not only prove useful in the future in clinical management
with respect to screening, surveillance, and prophylaxis, but also provide insight into
the genetic effects of particular mutations.
Trump D, Farren B, Wooding C, et al. Clinical studies of multiple endocrine
neoplasia type 1 (MEN 1). Q J Med. 1996;89:653-669.
Biochemical screening indicated that the penetrance of multiple endocrine neoplasia type 1
by the ages of 20, 35, and 50 years was 43%, 85%, and 94%, respectively, and that the
development of MEN 1 was confined to first-degree relatives in 91% of patients and to
second-degree relatives in 9% of patients. These findings have helped to define a proposed
screening program for MEN 1.
van Heerden JA, Young WF Jr, Grant CS, et al. Adrenal surgery for hypercortisolism:
surgical aspects. Surgery. 1995;117:466-472.
Although there is an effect of hypercortisolism on wound healing, infection, diabetes,
hypertension, coronary artery disease, and pulmonary embolism, it is possible to perform
adrenalectomy surgically with acceptable morbidity and mortality. These results may serve
as a standard against which laparoscopic adrenalectomy may be compared.
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