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Extralobar Sequestration Presenting as Massive Hemothorax
Extralobar Sequestration Presenting as Massive Hemothorax* Vered Avishai, MD; Eran Dolev, MD; Dov Weissberg, MD, FCCP; Liliana Zajdel, MD; and Israel E. Priel, MD, FCCP
Pulmonary extralohar sequestration is a rare anomaly, usually diagnosed during the first months of life. Acase of extralobar pulmonary sequestration in an adult, manifesting itself as massive hemothorax, is presented. (CHEST 1996; 109:843-45) CCAM=cystic adenomatoid malformation
I
Key words: cystic adenomatoid malformation; extralobar sequestration; hemothorax
sequestration is a relatively rare anomaly, diagB ronchial nosed usually at infancy. Of the two forms of bron-
FIGURE 2. Intercostal arteriography performed after the bleeding had been controlled. Tumor vessels draining into pulmonary vein are present.
chopulmonary sequestration, intralobar and extralobar, the latter is less frequently encountered. We describe an adult with extrapulmonary sequestration in whom massive spontaneous hemothorax was the presenting symptom. CASE REPORT
DISCUSSION
Balloon tamponade is a useful technique for treating hemoptysis. But inserting a balloon catheter through the working channel of a flexible bronchoscope, as previously reported, 1•2 has some demerits. First, insertion of a balloon catheter into the working channel reduces the suction capacity and thus makes it difficult to attain a good endoscopic view during the procedure, limiting the use of the technique to patients with slow bleeding. Second, a special balloon catheter with reattachable valve2 or complicated maneuver1 is necessary to remove the bronchoscope and then inflate the balloon. The modified insertion technique provides a better view during the procedure because only a guide wire needs to be inserted into the working channel, rendering minimal influence to suction capacity; tllis means that the technique could be applied to more massive bleeding cases. It requires no special catheter or complicated maneuver and is technically easy. We believe modified balloon tamponade is the preferred procedure in hemoptysis patients whose bleeding is located at the lobar level or beyond.
REFERENCES
1 Swersky RB, Ch
A 50-year-old healthy man was referred to the hospital because of a complaint of pain in the lower area of the left side of the c hest and the left area of the hypochondrium, 2 h following ergometry performed as part of a periodic checkup. Physical examination revealed only a slightly distended abdomen. The patient was admitted for observation. At the time of admission, the ECG was normal and remained so. A chest x-ray H.lm demonstrated a moderate pleural effusion on the left side. Twenty-four hours later, dyspnea and elevation of body temperature to 38°C developed, and a dull percussion sound with no air entry over the lower part of the left lung was noted. Another chest x-ray film indicated a significant increase in the amount of the pleural effusion. Closed-tube thoracostomy yielded non-clotting blood. Since the patient was experiencing chest pain and hemothorax on the le ft side, the possibility of dissection of the thoracic aorta was considered. Transesophageal echocardiography did not indicate any abnormal cardiac finding nor evidence of aortic dissection. However, a fairly large amount of pleural effusion was present on the left side, with a al rge partially moving mass within the fluid. ACT scan of the chest indicated the presence of a large left-sided pleural effusion surrounding a mass-like lesion, containing fluid with irregular, high-density foci in its periphery (Fig 1). Angiography of the thoracic aorta, celiac trunk, and left lower intercostal arteries showed no evidence of dissecting aneurysm, bleeding, or abnormal vessels supplying the lung tissue. The chest tube drained 2,800 mL bloody effusion within 48 h. Significant clinical improvement followed. However, following another episode of chest pain, asubsequent CT scan of the chest showed a marked increase in the size of the mass, with a minimal amount of fluid surrounding it. Subsequently, the patient underwent an explorative tl10racotomy. A cystic mass was found at the lower aspect of the left lung, *From the Departments of Internal Medicine E. (Drs. Avishai, Dolev, and Pnel), Thoracic Surgery (Dr. Weissberg), and Pathology (Dr. Zajdel), The Edith Wolfson Medical Center and Sackler School of Medicine, Tel Aviv University, Halon, Israel. CHEST I i09 I 3 I MARCH, i996
843
FIGURE 1. Noncontrast CT scan of the lower p art of the chest shows a large pleural effusion on the left. Enclosed in the fluid is a 5.2-cm inhomogenous, low-density, well-circumscribed round mass with high-density foci in its periphery. adherent to the diaphragm. It contained brown odorless fluid. Vascular connection to the thoracic aorta was found and divided. The el sion was resected. The pathologic finding consisted of a mass 10 em in diameter containing small cyst-like structures. The histologic features established the diagnosis o f xe:tralobar lung sequestration exhibiting cystic adenomatoid malformation (CCAM) (Fig 2). Postoperative recovery was uneventful. DISCUSSION
Extralobar sequestration, a n entity in which an banormal segment of the lung enclosed within its own pleural membrane is completely separated from the tracheobronchial tree, accounts for 25% of all pulmonary sequestrations, occurring predominantly in men with a r atio of 3 to 4: 1. 14 Its location, usually related to the left hemidiaphragm, may vary between the lower lobe and the diaphragm, in the mediastinum, within the lung, in the pleural or pericardia! spaces, or in the retroperitoneum .1·5 Arterial blood supply to the sequestered lung may originate from the tl1oracic or abdominal aorta (>80%); from smaller arteries, such as splenic, gastric, intercostal and subclavian (15%); or from branches of the pulmonary artery and systemic vessels (5%) . The venous drainage usually is connected to the azygos system; however, occasionally it can be connected t o tl1e intercostal, portal, esophageal, or adrenal veins. Pulmonary venous drainage also is possible.4 Intriguingly enough, the fe eding vessel was not identified on the angiogram, nor was it detected b y an independent retrospective review of tile films . One could only speculate whether tllis was the result of vasospasm, a sharp angle of exit from the aorta, or due to the fact that no technique is infallible. Extralobar s equestration, a d evelopmental lesion, usually presents during tile first 6 montlls of life (61 %), often witll dyspnea, cyanosis, and feeding difficulties.2 Recurrent pulmonary infections (as in intralobar sequestration) or gastrointestinal symptoms in the presence of a communication with the gastrointestinal tract (bronchopulmonary foregut malformation) are uncommon. Very few cases of CCAM-a developmental anomaly characterized b y multiple cysts of varying sizes as well as a 844
FIGURE 2. T!Yp : Cyst-like structures (hematoxylin-eosin, original xBO). Bottom: high-magnification of the ciliated pseudostratified epithelium covering a large cyst (hematoxylin-eosin, original x320). network of interconnecting spaces- were reported in tile adult. 6 Extralobar pulmonary sequestration tllat histologically displayed the features of CCAM type 2 was reported in 14 cases, 9 of which were thoracic and 5 of which w ere abdominal.7 Clinical features of extralobar sequestration alone or containing CCAM type 2 do not differ significantly.7 The occurrence of hemothorax unrelated to acute trauma is quite rare. The major diagnostic categories to be considered include defects of coagulation, malignant tumors, and primary vascular events-such as aortic dissection and pulmonary vascular bleeding. Rarely, infections, endometriosis, and costal exostoses can cause hemothorax.8 In tl1is patient, massive hemoiliorax was tile presenting sign of extralobar pulmonary sequestration. The diagnosis of extralobar sequestration in adults is railier difficult, perhaps because it is rarely thought of. 9 It is intriguing tllat of 66 patients witll extralobar sequestration who underwent surgery in tl1e series of Savic et al,2 a correct preoperative diagnosis was made only in 6. Spontaneous hemothorax from bronchopulmonary sequestration is exceedingly rare and only has been r eported in five cases. 10-12 To our best knowledge, all previously reported cases witll spontaneous hemothorax occurred with intralobar sequestration. In summary, acase of massive hemotllorax resulting from extralobar sequestration diagnosed at tlloracotomy was preSelected Reports
sented. Hemothorax in intralobar sequestration is exceedingly rare and in extralobar sequestration has not been previously described. ACKNOWLEDGMENTS: We thank D. Goncalves, MD, from the Department of Radiology, D . Harpaz, MD, from the Heart Institute at our hospital, and Z. Rubinstein, MD, from the Chaim Sheba Medical Center, Tel-Hashomer, Israel, for their help. REFERENCES
Fraser RG, Pare JAP, Pare PD, et al. Pulmonary abnormalities of developmental origin. In: Diagnosis of diseases of the chest. 3rd ed. Vol 2. Philadelphia: WB Saunders, 1989; 701-12 2 Savic B, Birtel FJ, Tholen W, et al. Lung sequestration: report of 7 cases and review of 540 published cases. Thorax 1979; 34:96-
101
3 Stocker JT. Congenital and developmental diseases. In: Dail DH, Hammer SP, eds. Pulmonary pathology New York: Springer Verlag, 1988; 53-5 4 Sade RM , Clouse M, Ellis HF. The spectrum of pulmonary sequestration. Ann Thorac Surg 1974; 18:644-55 .5 Felker RE , Tonkin ILD. Imaging of pulmonary sequestration. AJR 1990; 154:241-49 6 Avitabile AM, Hulnick DH, Greco MA, et al. Congenital cystic adenomatoid malformation of the lung in adults. Am J Surg Pathol 1984; 8:193-202 7 Aulicino MR, Reis ED, Dolgin SE, et al. Intra-abdominal pulmonary sequestration exhibiting congenital cystic adenomatoid malformation: rep01t of a case and review of the literature. Arch Pathol Lab Med 1994; 118:1034-37 8 Martinez FJ, Villanueva AG, Pickering R, etal. Spontaneous hemothorax: report of 6 cases and review of the literature. Medicine 1992; 71:354-66 9 Louie HW, Martin SM, Mulder DC. Pulmonary sequestration: 17-year experience at UCLA. Am Surg 1993; 59:801-05 10 Beau A,Collignon P, Guillamot M, et al. Hemothorax spontane, revelateur d'une sequestration pulmonaire. Ann Chir Infant 1966; 7:59-62 11 Laurin S, Aronson S, Schuler H, et al. Spontaneous hemothorax from bronchopulmonary sequestration. Pediatr Radiol1980; 10: 54-6 12 Zapatero J, Baamonde C, Bellone JM, et al. Hemothorax as rare presentation of intralobar sequestration. Scand J Thorac Cardiavase Surg 1983; 17:177-79
Use of Somatostatin Analog for Localization and Treatment of ACTH Secreting Bronchial Carcinoid Tumor* Sophie Christin-Maitre, MD; Nathalie Chabbert-Buffet, MD; Agnes Mure, MD; Rafik B01tkhris, MD; and Philippe B01tchard, MD, PhD
A 29-year-old woman presenting with an ectopic adrenocorticotropic hormone syndrome and a nodule of the upper lobe of the left lung was explored by in*From the Units of Endocrinology (Drs. Christin-Maitre, Chubbert-Buffet, and Bouchard) and-Nuclear Medicine (Dr. Mure), Hopital Saint Antoine, Paris, France; and Cinique Saint Augustin (Dr. Boukhris), Tunis, Tunisia. Manuscript received March 22, 1995; revision accepted August 30. Reprint requests: Dr. Bouchard, Hopital Saint-Antoine, 184 rue de Faubourg Saint-Antoine, 75012 Paris, France
dium Ill (1 11 In) octreotide scintigraphy. This showed a pathologic uptake by the nodule. Treatment with octreotide resulted in the rapid control of hypercortisolism prior to surgery. (CHEST 1996; 109:845-46)
Key words: bronchial carcinoid; Cushing's syndrome: ectopic ACTH secretion; somatostatin receptor scintigraphy tumors are responsible for 40% of cases of C arcinoid adrenocorticotropic hormone (ACTH) dependent
Cushing's syndrome_! Bronchial carcinoid tumors usually are small (0.3 to 1.3 em) and are difficult to localize. 2 In addition, hypercortisolism must be controlled prior to surgery. In this report, we describe, in a patient with ectopic ACTH syndrome, the rapid normalization of hypercortisolism by octreotide-only treatment, following the localization of a bronchial carcinoid tumor by octreotide scintigraphy. Octreotide is a somatostatin analog. This case report suggests that when a paraneoplasic ACTH -dependent hypercorticism is suspected, labeled pentetreotide scintigraphy may allow the exact localization of the tumor. Furthermore, the uptake of radioactive somatostatin (octreotide [Sandostatin; Sandoz; Bale, Switzerland]) suggests the possibility of treating patients with this drug. CASE REPORT
A 29-year-old woman was admitted to the hospital for the exploration of a rapidly evolving hypercortisolism, discovered as the patient complained of generalized edema, amenorrhea, and hypokalemia (2.8 mmoi!L). She weighed 80 kg and was 163-cm tall. She had a typical appearance of Cushing's syndrome. Biological evaluation confirmed the ACTH-dependent hypercortisolism. Results of a high-dose dexamethasone test were negative, and the plasma ACTH level did not increase after cortisol releasing factor administration, suggesting an ectopic ACTH secretion (Table 1).
Table !-Biological Evaluation Prior to Treatment, on Day 24 of SMS Treatment, and 1 Week and 2 Months Postsurgery* Values UFC, nmoll24 h Plasma cortisol, nmoi!L 8h 16 h Dexamethasone test 1 Plasma ACTH, pglmL, basal CRF testl Plasma LPH, pglmL
Initial Value
Treatment 1 Week Day 24 Postsurg
2 Months Postsurg
806 (<260)
13
24
33
1,493 (220-610) 1,103 (100-290) 1,166 214 (9-52) 246 1,674 (46-124)
116
123
151 211
101
18
32
66
* Surgery took place on treatment day 60. Normal value ranges are 1
shown in parentheses. UFC=urinary free cortisol; LPH=Iipotropin hormone; pg=picogram. 2-day high-dose dexamethason e suppression test (8 mg dexamethasone/d). Corticotropin-releasing factor, 100 pg administered intravenously. CHEST I 109 I 3 I MARCH, 1996
845
Pulmonary extralohar sequestration is a rare anomaly, usually diagnosed during the first months of life. Acase of extralobar pulmonary sequestration in an adult, manifesting itself as massive hemothorax, is presented. (CHEST 1996; 109:843-45) CCAM=cystic adenomatoid malformation
I
Key words: cystic adenomatoid malformation; extralobar sequestration; hemothorax
sequestration is a relatively rare anomaly, diagB ronchial nosed usually at infancy. Of the two forms of bron-
FIGURE 2. Intercostal arteriography performed after the bleeding had been controlled. Tumor vessels draining into pulmonary vein are present.
chopulmonary sequestration, intralobar and extralobar, the latter is less frequently encountered. We describe an adult with extrapulmonary sequestration in whom massive spontaneous hemothorax was the presenting symptom. CASE REPORT
DISCUSSION
Balloon tamponade is a useful technique for treating hemoptysis. But inserting a balloon catheter through the working channel of a flexible bronchoscope, as previously reported, 1•2 has some demerits. First, insertion of a balloon catheter into the working channel reduces the suction capacity and thus makes it difficult to attain a good endoscopic view during the procedure, limiting the use of the technique to patients with slow bleeding. Second, a special balloon catheter with reattachable valve2 or complicated maneuver1 is necessary to remove the bronchoscope and then inflate the balloon. The modified insertion technique provides a better view during the procedure because only a guide wire needs to be inserted into the working channel, rendering minimal influence to suction capacity; tllis means that the technique could be applied to more massive bleeding cases. It requires no special catheter or complicated maneuver and is technically easy. We believe modified balloon tamponade is the preferred procedure in hemoptysis patients whose bleeding is located at the lobar level or beyond.
REFERENCES
1 Swersky RB, Ch
A 50-year-old healthy man was referred to the hospital because of a complaint of pain in the lower area of the left side of the c hest and the left area of the hypochondrium, 2 h following ergometry performed as part of a periodic checkup. Physical examination revealed only a slightly distended abdomen. The patient was admitted for observation. At the time of admission, the ECG was normal and remained so. A chest x-ray H.lm demonstrated a moderate pleural effusion on the left side. Twenty-four hours later, dyspnea and elevation of body temperature to 38°C developed, and a dull percussion sound with no air entry over the lower part of the left lung was noted. Another chest x-ray film indicated a significant increase in the amount of the pleural effusion. Closed-tube thoracostomy yielded non-clotting blood. Since the patient was experiencing chest pain and hemothorax on the le ft side, the possibility of dissection of the thoracic aorta was considered. Transesophageal echocardiography did not indicate any abnormal cardiac finding nor evidence of aortic dissection. However, a fairly large amount of pleural effusion was present on the left side, with a al rge partially moving mass within the fluid. ACT scan of the chest indicated the presence of a large left-sided pleural effusion surrounding a mass-like lesion, containing fluid with irregular, high-density foci in its periphery (Fig 1). Angiography of the thoracic aorta, celiac trunk, and left lower intercostal arteries showed no evidence of dissecting aneurysm, bleeding, or abnormal vessels supplying the lung tissue. The chest tube drained 2,800 mL bloody effusion within 48 h. Significant clinical improvement followed. However, following another episode of chest pain, asubsequent CT scan of the chest showed a marked increase in the size of the mass, with a minimal amount of fluid surrounding it. Subsequently, the patient underwent an explorative tl10racotomy. A cystic mass was found at the lower aspect of the left lung, *From the Departments of Internal Medicine E. (Drs. Avishai, Dolev, and Pnel), Thoracic Surgery (Dr. Weissberg), and Pathology (Dr. Zajdel), The Edith Wolfson Medical Center and Sackler School of Medicine, Tel Aviv University, Halon, Israel. CHEST I i09 I 3 I MARCH, i996
843
FIGURE 1. Noncontrast CT scan of the lower p art of the chest shows a large pleural effusion on the left. Enclosed in the fluid is a 5.2-cm inhomogenous, low-density, well-circumscribed round mass with high-density foci in its periphery. adherent to the diaphragm. It contained brown odorless fluid. Vascular connection to the thoracic aorta was found and divided. The el sion was resected. The pathologic finding consisted of a mass 10 em in diameter containing small cyst-like structures. The histologic features established the diagnosis o f xe:tralobar lung sequestration exhibiting cystic adenomatoid malformation (CCAM) (Fig 2). Postoperative recovery was uneventful. DISCUSSION
Extralobar sequestration, a n entity in which an banormal segment of the lung enclosed within its own pleural membrane is completely separated from the tracheobronchial tree, accounts for 25% of all pulmonary sequestrations, occurring predominantly in men with a r atio of 3 to 4: 1. 14 Its location, usually related to the left hemidiaphragm, may vary between the lower lobe and the diaphragm, in the mediastinum, within the lung, in the pleural or pericardia! spaces, or in the retroperitoneum .1·5 Arterial blood supply to the sequestered lung may originate from the tl1oracic or abdominal aorta (>80%); from smaller arteries, such as splenic, gastric, intercostal and subclavian (15%); or from branches of the pulmonary artery and systemic vessels (5%) . The venous drainage usually is connected to the azygos system; however, occasionally it can be connected t o tl1e intercostal, portal, esophageal, or adrenal veins. Pulmonary venous drainage also is possible.4 Intriguingly enough, the fe eding vessel was not identified on the angiogram, nor was it detected b y an independent retrospective review of tile films . One could only speculate whether tllis was the result of vasospasm, a sharp angle of exit from the aorta, or due to the fact that no technique is infallible. Extralobar s equestration, a d evelopmental lesion, usually presents during tile first 6 montlls of life (61 %), often witll dyspnea, cyanosis, and feeding difficulties.2 Recurrent pulmonary infections (as in intralobar sequestration) or gastrointestinal symptoms in the presence of a communication with the gastrointestinal tract (bronchopulmonary foregut malformation) are uncommon. Very few cases of CCAM-a developmental anomaly characterized b y multiple cysts of varying sizes as well as a 844
FIGURE 2. T!Yp : Cyst-like structures (hematoxylin-eosin, original xBO). Bottom: high-magnification of the ciliated pseudostratified epithelium covering a large cyst (hematoxylin-eosin, original x320). network of interconnecting spaces- were reported in tile adult. 6 Extralobar pulmonary sequestration tllat histologically displayed the features of CCAM type 2 was reported in 14 cases, 9 of which were thoracic and 5 of which w ere abdominal.7 Clinical features of extralobar sequestration alone or containing CCAM type 2 do not differ significantly.7 The occurrence of hemothorax unrelated to acute trauma is quite rare. The major diagnostic categories to be considered include defects of coagulation, malignant tumors, and primary vascular events-such as aortic dissection and pulmonary vascular bleeding. Rarely, infections, endometriosis, and costal exostoses can cause hemothorax.8 In tl1is patient, massive hemoiliorax was tile presenting sign of extralobar pulmonary sequestration. The diagnosis of extralobar sequestration in adults is railier difficult, perhaps because it is rarely thought of. 9 It is intriguing tllat of 66 patients witll extralobar sequestration who underwent surgery in tl1e series of Savic et al,2 a correct preoperative diagnosis was made only in 6. Spontaneous hemothorax from bronchopulmonary sequestration is exceedingly rare and only has been r eported in five cases. 10-12 To our best knowledge, all previously reported cases witll spontaneous hemothorax occurred with intralobar sequestration. In summary, acase of massive hemotllorax resulting from extralobar sequestration diagnosed at tlloracotomy was preSelected Reports
sented. Hemothorax in intralobar sequestration is exceedingly rare and in extralobar sequestration has not been previously described. ACKNOWLEDGMENTS: We thank D. Goncalves, MD, from the Department of Radiology, D . Harpaz, MD, from the Heart Institute at our hospital, and Z. Rubinstein, MD, from the Chaim Sheba Medical Center, Tel-Hashomer, Israel, for their help. REFERENCES
Fraser RG, Pare JAP, Pare PD, et al. Pulmonary abnormalities of developmental origin. In: Diagnosis of diseases of the chest. 3rd ed. Vol 2. Philadelphia: WB Saunders, 1989; 701-12 2 Savic B, Birtel FJ, Tholen W, et al. Lung sequestration: report of 7 cases and review of 540 published cases. Thorax 1979; 34:96-
101
3 Stocker JT. Congenital and developmental diseases. In: Dail DH, Hammer SP, eds. Pulmonary pathology New York: Springer Verlag, 1988; 53-5 4 Sade RM , Clouse M, Ellis HF. The spectrum of pulmonary sequestration. Ann Thorac Surg 1974; 18:644-55 .5 Felker RE , Tonkin ILD. Imaging of pulmonary sequestration. AJR 1990; 154:241-49 6 Avitabile AM, Hulnick DH, Greco MA, et al. Congenital cystic adenomatoid malformation of the lung in adults. Am J Surg Pathol 1984; 8:193-202 7 Aulicino MR, Reis ED, Dolgin SE, et al. Intra-abdominal pulmonary sequestration exhibiting congenital cystic adenomatoid malformation: rep01t of a case and review of the literature. Arch Pathol Lab Med 1994; 118:1034-37 8 Martinez FJ, Villanueva AG, Pickering R, etal. Spontaneous hemothorax: report of 6 cases and review of the literature. Medicine 1992; 71:354-66 9 Louie HW, Martin SM, Mulder DC. Pulmonary sequestration: 17-year experience at UCLA. Am Surg 1993; 59:801-05 10 Beau A,Collignon P, Guillamot M, et al. Hemothorax spontane, revelateur d'une sequestration pulmonaire. Ann Chir Infant 1966; 7:59-62 11 Laurin S, Aronson S, Schuler H, et al. Spontaneous hemothorax from bronchopulmonary sequestration. Pediatr Radiol1980; 10: 54-6 12 Zapatero J, Baamonde C, Bellone JM, et al. Hemothorax as rare presentation of intralobar sequestration. Scand J Thorac Cardiavase Surg 1983; 17:177-79
Use of Somatostatin Analog for Localization and Treatment of ACTH Secreting Bronchial Carcinoid Tumor* Sophie Christin-Maitre, MD; Nathalie Chabbert-Buffet, MD; Agnes Mure, MD; Rafik B01tkhris, MD; and Philippe B01tchard, MD, PhD
A 29-year-old woman presenting with an ectopic adrenocorticotropic hormone syndrome and a nodule of the upper lobe of the left lung was explored by in*From the Units of Endocrinology (Drs. Christin-Maitre, Chubbert-Buffet, and Bouchard) and-Nuclear Medicine (Dr. Mure), Hopital Saint Antoine, Paris, France; and Cinique Saint Augustin (Dr. Boukhris), Tunis, Tunisia. Manuscript received March 22, 1995; revision accepted August 30. Reprint requests: Dr. Bouchard, Hopital Saint-Antoine, 184 rue de Faubourg Saint-Antoine, 75012 Paris, France
dium Ill (1 11 In) octreotide scintigraphy. This showed a pathologic uptake by the nodule. Treatment with octreotide resulted in the rapid control of hypercortisolism prior to surgery. (CHEST 1996; 109:845-46)
Key words: bronchial carcinoid; Cushing's syndrome: ectopic ACTH secretion; somatostatin receptor scintigraphy tumors are responsible for 40% of cases of C arcinoid adrenocorticotropic hormone (ACTH) dependent
Cushing's syndrome_! Bronchial carcinoid tumors usually are small (0.3 to 1.3 em) and are difficult to localize. 2 In addition, hypercortisolism must be controlled prior to surgery. In this report, we describe, in a patient with ectopic ACTH syndrome, the rapid normalization of hypercortisolism by octreotide-only treatment, following the localization of a bronchial carcinoid tumor by octreotide scintigraphy. Octreotide is a somatostatin analog. This case report suggests that when a paraneoplasic ACTH -dependent hypercorticism is suspected, labeled pentetreotide scintigraphy may allow the exact localization of the tumor. Furthermore, the uptake of radioactive somatostatin (octreotide [Sandostatin; Sandoz; Bale, Switzerland]) suggests the possibility of treating patients with this drug. CASE REPORT
A 29-year-old woman was admitted to the hospital for the exploration of a rapidly evolving hypercortisolism, discovered as the patient complained of generalized edema, amenorrhea, and hypokalemia (2.8 mmoi!L). She weighed 80 kg and was 163-cm tall. She had a typical appearance of Cushing's syndrome. Biological evaluation confirmed the ACTH-dependent hypercortisolism. Results of a high-dose dexamethasone test were negative, and the plasma ACTH level did not increase after cortisol releasing factor administration, suggesting an ectopic ACTH secretion (Table 1).
Table !-Biological Evaluation Prior to Treatment, on Day 24 of SMS Treatment, and 1 Week and 2 Months Postsurgery* Values UFC, nmoll24 h Plasma cortisol, nmoi!L 8h 16 h Dexamethasone test 1 Plasma ACTH, pglmL, basal CRF testl Plasma LPH, pglmL
Initial Value
Treatment 1 Week Day 24 Postsurg
2 Months Postsurg
806 (<260)
13
24
33
1,493 (220-610) 1,103 (100-290) 1,166 214 (9-52) 246 1,674 (46-124)
116
123
151 211
101
18
32
66
* Surgery took place on treatment day 60. Normal value ranges are 1
shown in parentheses. UFC=urinary free cortisol; LPH=Iipotropin hormone; pg=picogram. 2-day high-dose dexamethason e suppression test (8 mg dexamethasone/d). Corticotropin-releasing factor, 100 pg administered intravenously. CHEST I 109 I 3 I MARCH, 1996
845