ABSTRACT
A prospective study of 149 patients suffering from adenocarcinoma of unknown primary site (ACUPS) of bone was carried out. The tumors are classified as 63 extraspinal, 67 spinal and 19 mixed involvement. Upon meticulous physical examination, Virchow's node was detected in 15 cases, rectal shelf in 11 cases and hepatomegaly in 44 cases. Blood chemistry showed elevation of alkaline phosphatase (> 3 sigma units) in 98 cases and chest roentgenogram showed pulmonary lesions in 23 cases. Treatment was surgery and radiotherapy in 64 and two cases respectively. In all of these patients histological findings of the biopsy or resection specimen had confirmed the diagnosis of adenocarcinoma. Among 124 evaluable patients, overall survival was analysed using Kaplan-Meier life table analysis. Survival rates at one, two and four months after diagnosis were 80.7, 60.5 and 25 per cent with a mean and median survival times of 90 and 77 days respectively. Statistical analysis was also performed to ascertain the prognostic importance of the following variables: age, gender, Virchow's node, rectal shelf, hepatomegaly, serum alkaline phosphatase, pulmonary lesion, and multiplicity and site of the osseous lesions. The presence of pulmonary lesion or hepatomegaly significantly produced unfavorable impact on the duration of survival (p = 0.0004 and 0.0150, respectively) while other remaining factors had not.
Subject(s)
Adenocarcinoma/mortality , Adult , Aged , Bone Neoplasms/mortality , Female , Follow-Up Studies , Humans , Male , Middle Aged , Neoplasms, Unknown Primary/mortality , Prognosis , Prospective Studies , Spinal Neoplasms/mortality , Survival RateABSTRACT
Maffucci's syndrome is a particularly rare clinical form of mesodermal dysplasia. It consists of angiomatosis, chondromatosis and sometimes dyschromic cutaneous lesions. The syndrome is usually associated with a high incidence of malignancy, originating primarily from mesodermal derivatives. The present case is the second in the literature in which the syndrome is associated with a frontal lobe astrocytoma. This patient and those reported in the literature, suggest the potential involvement of non-mesodermal tumors in the Maffucci's syndrome.