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Long acting somatostatin treatment of paraneoplastic Cushing's syndrome in a case of Zollinger-Ellison syndrome.

Cushing's syndrome, caused by ectopic ACTH production during Zollinger-Ellison syndrome, raises difficult therapeutic problems. We report a case of clinical and biological efficacy of long acting somatostatin (SMS) in this condition. In a short term study with 200 micrograms SMS bid, symptoms of hypercorticism disappeared while cortisol and ACTH serum concentrations fell below the normal values. Longterm treatment was instituted with 50 micrograms SMS bid. Excellent clinical efficacy as well as normal cortisol and ACTH serum concentrations were maintained during the nine month follow up. Lipotrophic hormone (LPH) serum concentration remained raised. No decrease in size of hepatic metastases was observed. Long acting somatostatin analogues may be useful in endocrine paraneoplastic syndromes.

ACTH Syndrome, Ectopic↗

Ectopic adrenocorticotrophic (ACTH) syndrome and small cell carcinoma of the lung-assessment of clinical implications in patients on combination chemotherapy.

Small cell carcinoma of the lung is the tumor most commonly associated with ectopic ACTH production and hypercortisolism. The relationship between this paraneoplastic endocrine syndrome and the clinical course of the tumor is examined in this review of patients with the ectopic ACTH syndrome and small cell carcinoma seen at The Johns Hopkins Oncology Center between 1973 and 1979. Five of 157 (3.2%) patients with small cell carcinoma were clinically diagnosed as having the ectopic ACTH syndrome. The onset of this endocrine syndrome appeared to coincide with a more aggressive phase of the course of small cell carcinoma. Further analysis of these cases suggests that the development of the ectopic ACTH syndrome may reflect changes in cell populations within the tumor and/or alterations in tumor behavior with time and perhaps with the effects of drug therapy.

ACTH Syndrome, Ectopic↗

[Schwartz-Bartter syndrome. Presentation of a sub-glottal small cell laryngeal carcinoma (author's transl)].

Inappropriate secretion of antidiuretic hormone or Schwartz-Bartter syndrome is considered to be a paraneoplastic endocrine syndrome, most frequently associated with a small cell anaplastic carcinoma of the lung. The authors present a case of sub-glottal laryngeal carcinoma discovered several months after the onset of clinical (disturbances of conscious level) and laboratory (hyponatraemia, hypochloraemia, blood hypo-osmolarity which preservation of urinary sodium output) manifestations which form the original features of this syndrome, the criteria of which are reviewed. The y conclude by the need for complete examination, not excluding unusual sites of primary tumour.

Aged↗

The aggressive surgical management of hypercalcemia due to ectopic parathormone production.

A patient with slowly progressive metastatic renal cell carcinoma had life-threatening hypercalcemia secondary to ectopic parathormone production. Aggressive surgical debulking resulted in prolonged control of the hypercalcemia. This case illustrates the value of this surgery in the control of paraneoplastic endocrine syndromes refractory to medical management in patients with slow-growing metastatic disease.

Adenocarcinoma↗

[Gynecomastia and cancer of the bladder: an ectopic secretion of chorionic gonadotropin hormone].

Among the paraneoplastic endocrine syndromes, gynecomastia induced by an ectopic production of human chorionic gonadotrophin remains infrequent. The authors report the case of a fifty-year-old man presenting with a bilateral gynecomastia which had been evolving over three months. The etiological investigation was rapidly guided by a macroscopic hematuria. Intravenous nephro-urotomographies visualized a filling defect in the bladder, and a grade III stage A urothelial carcinoma was completely resected cystoscopically, leading to progressive disappearance of the gynecomastia. Hormonal investigation revealed an ectopic production of HCG, inducing hyperestrogenia.

Chorionic Gonadotropin↗

[Paraneoplastic syndromes (1)].

Although not rare, paraneoplastic syndromes develop in a minority of cancer patients. Their exact frequency seems to be underestimated. These syndromes are hereby described. Their clinical usefulness is of great importance: they can be the first sign of a malignancy and can be used as tumor markers. The diagnosis of paraneoplastic syndromes is often difficult. A "diagnosis score" is proposed in order to make the diagnosis easier.

Carcinoid Heart Disease↗

[Etiology of ectopic hormone producing tumors].

Inappropriate secretion of peptide hormones and other associated proteins by tumors is the most common cause of paraneoplastic syndromes. Sensitive assay techniques have permitted detection of polypeptide hormone secretion by tumors even in the absence of clinically overt syndromes. Moreover, it has been demonstrated that most or all nonendocrine tissues produce small amounts of a variety of peptide hormones and hormone precursors. Nevertheless, the term ectopic hormone syndromes occurring with nonendocrine neoplasms is well established and widely used. We summarize etiology of 'classic' ectopic hormone producing tumors to help establish the diagnosis of paraneoplastic humoral syndromes.

ACTH Syndrome, Ectopic↗

[Paraneoplastic syndromes: a review].

Modern oncology often obtains good results against earlier neoplasms, whilst it's still in difficulties against the advanced ones. The knowledge of paraneoplastic syndromes is crucial both to cure patients and to do an earlier diagnosis. When we recognize a paraneoplastic syndrome that comes before the clinic beginning of a neoplasm, perhaps we save a life. This review discusses all the main paraneoplastic syndromes, focusing mainly on their clinical aspect and reminding the most commonly associated cancers.

ACTH Syndrome, Ectopic↗

Paraneoplastic syndromes in hypernephroma.

It is noted that while a wide variety of syndromes have been associated with hypernephroma in the clinical literature, there is clear understanding of the pathophysiology of these effects only in the cases of the endocrine disorders where direct tumor production of hormone can be demonstrated in vitro. Furthermore, this knowledge has done little to alter the care of patients with the disease, except for indications that indomethacin might be of benefit in some patients with hypercalcemia and that one might consider the use of converting enzyme inhibitors in patients with hypernephroma and hypertension. The overall approach to the disease is still surgical. Resection of the tumor also removes the paraneoplastic syndrome. Persistence or recurrence of a syndrome suggests the continued presence of the neoplasm, with the considerations for prognosis which that fact entails. To that degree, at least, these conditions are useful as tumor markers, but such use is limited because they are inconsistent. Further studies of pathophysiology of paraneoplastic syndromes will lead to better understanding of processes of cell differentiation and regulation, and possibly better ways to manage the patients in which they occur.

Carcinoma, Renal Cell↗

Non-small-cell lung cancer with multiple paraneoplastic syndromes.

We describe the case of a patient with multiple paraneoplastic syndromes, six in total, associated with a non-small-cell cancer of the lung. In this single patient we found hypertrophic pulmonary osteoarthropathy, hyperkeratosis of palms and soles, erythema annulare centrifugum, syndrome of inappropriate secretion of antidiuretic hormone (SIADH), and ectopic andrenocorticotrophic hormone (ACTH) and calcitonin production.

ACTH Syndrome, Ectopic↗