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INTRODUCTION

A carcinoid tumor occurring in the endometrium has been documented in the literature, but there is no report in regard to carcinoid tumor metastasis to endometrium.

PRESENTATION OF CASE

We report a case of a malignant carcinoid metastasis to an endometrial polyp. Patient underwent hysteroscopy, and polypectomy. The pathology demonstrated an endometrial polyp containing a 4 mm x 5 mm nodule of metastatic carcinoid tumor, consistent with metastasis from patient's known pulmonary carcinoid. The tumor was morphologically similar to the tumors of the right lung, with similar immune-profile.

DISCUSSION

This patient presented with a suspicious pelvic ultrasound. Due to her age, the first priority was to exclude uterine cancer. The endometrial polyp, which was found, had a small focus of metastatic carcinoid tumor. To the best of our knowledge, this finding has not been previously recorded in the literature. Our patient also had a history of metastatic carcinoid tumor to breast. This finding is also very uncommon.

CONCLUSION

This is the first case in the literature described a malignant carcinoid metastasis to an endometrial polyp.

Carcinoids are neuroendocrine tumors mainly involving the gastrointestinal tract, lungs and bronchi. They were considered benign with slow growth, but they can be malignant in a substantial percentage of patients (metastasizing to liver, bones, skin, etc). Endocrine activity results in carcinoid syndrome. Proximal myopathy has been reported in 7% of patients with carcinoid syndrome. Bronchopulmonary and thymic carcinoids producing adrenocorticotropic hormone can cause Cushing’s syndrome, a main feature of which is myopathy. There are a few reports of carcinoids associated with paraneoplastic neurological syndromes, including neuropathy. We hereby present an extremely rare case of a primary lung carcinoid presented with paraparesis due to polyneuropathy, and review the relevant literature. To the best of our knowledge there is no similar previous report. Complete resolution of paraparesis after excision of the lung carcinoid suggests paraneoplastic neurological syndrome.

Background

Carcinoid tumors are characteristically low grade malignant neoplasms with neuroendocrine differentiation that arise in various body sites, most commonly the lung and gastrointestinal tract, but less frequently the kidneys, breasts, ovaries, testes, prostate and other locations. We report a case of a carcinoid of renal origin with synchronous single liver metastases on radiological studies.

Case presentation

A 45 year-old patient who presented with abdominal pain was found on CT scan to have lesions in the right ovary, right kidney, and left hepatic lobe. CA-125, CEA, and CA 19-9 were within normal limits, as were preoperative liver function tests and renal function. Biopsy of the liver mass demonstrated metastatic neuroendocrine tumor. At laparotomy, the patient underwent total abdominal hysterectomy with bilateral salpingo-oophorectomy, radical right nephrectomy with lymphadenectomy, and left hepatectomy. Pathology evaluation reported a right ovarian borderline serous tumor, well-differentiated neuroendocrine carcinoma of the kidney (carcinoid) with 2 positive retroperitoneal lymph nodes, and a single liver metastasis. Immunohistochemistry revealed that this lesion was positive for synaptophysin and CD56, but negative for chromogranin as well as CD10, CD7, and CD20, consistent with a well-differentiated neuroendocrine tumor. She is doing well one year after her initial surgery, with no evidence of tumor recurrence.

Conclusion

Early surgical intervention, together with careful surveillance and follow-up, can achieve successful long-term outcomes in patients with this rare malignancy.

Background

Metastatic breast carcinoids are rare neoplasms. They can be mistaken for primary breast carcinoma both clinically and radiologically, even with known history of carcinoid tumor elsewhere in the body.

Case presentation

We report a case of unilateral breast metastasis from carcinoid tumor of the small intestine in a 52-year-old woman who was successfully treated by lumpectomy and radiation therapy. An extensive review of the literature reveals only a few cases of metastatic carcinoid to the breast from small intestinal primaries.

Conclusion

Clinical suspicion for metastasis should be high in a patient with breast mass and history of known carcinoid elsewhere in the body. Lumpectomy alone may be effective in these patients. Mastectomy and especially axillary dissection could be avoided. Their histological appearance may mimic ductal adenocarcinoma of the breast. However, the distinction is important due to differences in management and prognosis.

Carcinoid tumours, most frequently reported in the gastrointestinal and respiratory tracts, are exceedingly rare primary renal cancers. Few cases have been published to date. To our knowledge, we report the first case of a primary carcinoid tumour of the kidneys involving the inferior vena cava. We treated a 58-year-old woman with an open radical nephrectomy and cavotomy with thrombectomy. We describe the presentation, investigations and pathology results. We discuss the current experience with carcinoid tumours as a literature review relating to the diagnosis of the disease and the prognosis of patients with this neoplasm. Localized carcinoid tumours of the kidneys, including those involving the vena cava, can be successfully treated with surgical excision.

Background

Carcinoids are neuroendocrine tumors involving the gastrointestinal tract, lungs and bronchi. They are usually benign with slow growth, but they can be malignant (metastasizing to liver, lungs, bones, skin, etc). Endocrine activity results in carcinoid syndrome: Intestinal carcinoid [5-hydroxytryptamine (serotonin) to portal circulation]: abdominal cramps, diarrhea, bronchospasm, face/chest cyanotic flushes, and right-sided stenotic cardiac lesions. Lung carcinoid (histamine and 5-hydroxytryptophan to systemic circulation): red facial flush/oedema, salivation, lachrymation, potentially tachycardia, fever, anxiety, tremor, hypotension, left-sided cardiac lesions). We present an extremely rare case of primary lung carcinoid presented with paraparesis.

Patient and methods

A 69 year old man developed progressively deteriorating bilateral proximal muscular weakness of the legs that within 6 months led to paraparesis (inability to stand up and walk) with preserved sensation. Electromyography showed polyneuropathy. History, clinical and laboratory data excluded diabetic mellitus, hypothyroidism, renal/liver failure, cachexia, alcohol abuse, chemotherapy, drug or other toxicity. Magnetic resonance imaging excluded spinal cord or spinal nerve root compression. Computed tomography revealed a small peripheral paracardiac right-middle lobe tumor (maximal diameter: 1.8cm). Thoracoscopic wedged resection was performed (endo-stappler). Histology revealed lung carcinoid of low mitotic index (1-2%).

Results

The patient had an uneventful recovery, was discharged on the 5th postoperative day to a rehabilitation centre, where he remained for 3 months. Progressive motor function improvement was noted; paraparesis resolved completely within 6 months. At 1-year follow-up normal respiratory and neurological status was noted, in the absence of carcinoid recurrence or metastasis.

Conclusions

Myopathy is common in endocrine disease and cancer can have endocrine and metabolic activities and cause neurological complications. Encephalopathy, myelopathy, and more often motor and sensory neuropathy, myopathy, or neuromyopathy of central distribution may occur in paraneoplastic syndromes. Neuromyopathy has been reported in 14% of lung cancer patients. There are reports of recurrent facial palsy in metastatic thymic carcinoid, proximal muscle weakness in ACTH-releasing thymic carcinoid, an ACTH-releasing bronchial carcinoid, and paraparesis 40 years after pneumonectomy for carcinoid adenoma induced by inflammatory contents of the pneumonectomy cavity. To the best of our knowledge there is no report of paraparesis induced by primary pulmonary carcinoid. Complete resolution after excision suggests “paraneoplastic” syndrome.

Background

Rectal carcinoids comprise only about 1% of all anorectal neoplasms. In addition, ganglioneuroma of the gastrointestinal tract is a rare tumor composed ganglion cells, nerve fibers, and supporting cells. Multiple carcinoid tumors with diffuse ganglioneuromatosis limited to the rectum are quite unusual.

Case presentation

A 69-year-old man was referred to us because of about 100 small submucosal rectal tumors. He underwent abdominoperineal resection. Pathology revealed carcinoid tumors for about 30 submucosal nodules and diffuse ganglioneuromotosis. To date (6 months later) he remains well with no recurrence.

Conclusion

Although the optimal treatment for the multiple rectal carcinoids remains to be clearly established, it is believed that not all patients with multiple rectal carcinoids (measuring less than 1 cm in diameter) need to have a radical operation. However, the treatment plan for each case should be individualized and a careful follow-up is mandatory.

Three cases of carcinoid tumour of the stomach associated with primary hyperparathyroidism had the clinical and pathological features of a pluriglandular syndrome. Two of the patients showed multiple small polypoid carcinoids in the non-antral stomach, in conjunction with a parathyroid adenoma in one and parathyroid hyperplasia in the other case. One of these patients was also suffering from pernicious anaemia. A third patient had a large metastasising carcinoid arising in the gastric body and a parathyroid adenoma. Immunohistochemical stains for PGP 9.5 were positive in the carcinoids of all three cases. In all cases the carcinoids showed immunoreactivity for gastrin. A positive family history of endocrine hyperplasia and neoplasia was established in one case. It is suggested that patients with gastrointestinal carcinoids and their families should be evaluated for hyperparathyroidism, and patients with hyperparathyroidism presenting with upper gastrointestinal symptoms should undergo endoscopy to rule out gastric carcinoid tumours.

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Background

Malignant transformation of mature cystic teratoma is a rare complication. While any of the constituent tissues of a teratoma has the potential to undergo malignant transformation, squamous cell carcinoma is the most commonly associated malignancy. Renal carcinoid tumors are rare and frequently associated with horseshoe kidney and renal teratoma. Renal teratoma rarely presents together with carcinoid tumor or adenocarcinoma. To the best of our knowledge, there has never been a report of renal teratoma coexisting with both carcinoid tumor and adenocarcinoma.

Methods

Here, we present a unique and first case of synchronous primary carcinoid tumor and moderately differentiated adenocarcinoma arising within mature cystic teratoma of horseshoe kidney in a 50-year-old female. Lumbar spine X-ray, done for her complaint of progressive chronic low back pain, accidentally found a large calcification overlying the lower pole of the right kidney. Further radiologic studies revealed horseshoe kidney and a large multiseptated cystic lesion immediately anterior to the right renal pelvis with central calcification and peripheral enhancement. She underwent right partial nephrectomy.

Results

Macroscopically, the encapsulated complex solid and multiloculated cystic tumor with large calcification, focal thickened walls and filled with yellow-tan gelatinous material. Microscopically, the tumor showed coexistent mature cystic teratoma, moderately differentiated adenocarcinoma and carcinoid tumor. Immunohistochemically, alpha-methylacyl-coenzyme A-racemase, calretinin, CD10 and thyroid transcription factor-1 were negative in all the three components of the tumor. The teratomatous cysts lined by ciliated epithelium showed strong staining for cytokeratin 7 and pancytokeratin, and those lined by colonic-like epithelium showed strong staining for CDX2, cytokeratin 20 and pancytokeratin, but both were negative for calretinin. Additionally, the teratomatous cyst wall showed strong staining for smooth muscle actin, and weak staining for carbonic anhydrase IX, CD99, chromogranin and synaptophysin. The adenocarcinoma component was strongly positive for cytokeratin 7 and pancytokeratin, weakly positive for synaptophysin and CD56, and negative for carbonic anhydrase IX, CD99, CDX2, chromogranin, cytokeratin 20 and smooth muscle actin. The carcinoid tumor component was strongly positive for CD56, chromogranin and synaptophysin, weakly positive for pancytokeratin, and negative for carbonic anhydrase IX, CD99, CDX2, cytokeratin 7, cytokeratin 20 and smooth muscle actin. She received no adjuvant therapy and is alive without evidence of disease six months after diagnosis and surgery.

Conclusion

This unique and first case herein presented with synchronous primary carcinoid tumor and primary adenocarcinoma arising within mature cystic teratoma of horseshoe kidney emphasizes the need for thorough sectioning and entire submission for histologic evaluation of mature cystic teratomas, in order to avoid missing multiple additional histogenetically distinct neoplasms.

Carcinoid tumors are the most common neuroendocrine tumors. Gastric carcinoids represent 2% of all carcinoids and 1% of all gastric masses. Due to the widespread use of Esophagogastroduodenoscopy for evaluating a variety of upper gastrointestinal symptoms, the detection of early gastric carcinoids has increased. We highlight an alternative management of a young patient with recurrent type 1 gastric carcinoids with greater than 5 lesions, as well as lesions intermittently greater than 1 cm. Gastric carcinoids have a variable presentation and clinical course that is highly dependent on type. Type 1 gastric carcinoids are usually indolent and have a metastasis rate of less than 2%, even with tumors larger than 2 cm. There are a number of experts as well as organizations that recommend endoscopic resection for all type 1 gastric carcinoid lesions less than 1 cm, with a follow-up every 6-12 mo. They also recommend antrectomy for type 1 gastric carcinoids with greater than 5 lesions, lesions 1 cm or greater, or refractory anemia. However, the American Society of Gastrointestinal Endoscopy guidelines state that type 1 gastric carcinoid surveillance is controversial based on the evidence and could not make an evidence-based position statement on the best treatment modality. Our report illustrates a rare cause of iron deficiency anemia in a young male (without any medical history) due to multiple recurrent gastric carcinoid type 1 lesions in the setting of atrophic gastritis causing hypergastrinemia, and in the absence of a vitamin B12 deficiency. Gastric carcinoid type 1 can present in young males without an autoimmune history, despite the known predilection for women aged 50 to 70 years. Type 1 gastric carcinoids can be managed by endoscopic resection in patients with greater than 5 lesions, even with lesions larger than 1 cm. This course of treatment enabled the avoidance of early antrectomy in our patient, who expressed a preference against more invasive measures at his young age.

Introduction. Carcinoid tumors are low-grade tumors originating from endoderm and mostly involving the gastrointestinal system. However; they may be seen in any site within the gastrointestinal system. Case Presentation. A 69-year-old female patient. The results of blood tests were observed to be consistent with obstructive jaundice. A mass appearance was not encountered on tomographic examination. Papilla that was tumor-like macroscopically was seen in the second part of the duodenum in diagnostic endoscopy. Pylorus—preserving pancreaticoduodenectomy surgical procedure was applied. On pathological examination of the mass, a tumoral mass was detected in ampulla vateri localization, 1.5 × 1 × 0.8 cm in size, which, in immunohistochemical staining, was evaluated as a neuroendocrine tumor. Also, Metastasis was observed. Conclusion. The rarest type of carcinoid tumor is ampullary located carcinoid tumor, and tumor size is not a reliable indicator for tumor aggressivity in ampullary carcinoid tumors.

Extrapulmonary poorly differentiated neuroendocrine carcinomas can originate in the gastrointestinal tract, bladder, cervix, and prostate. These high-grade malignancies are characterized by aggressive histological features (high mitotic rate, extensive necrosis, and nuclear atypia) and a poor clinical prognosis. They are infrequently associated with secretory hormonal syndromes (such as the carcinoid syndrome) and rarely express somatostatin receptors.

Most poorly differentiated neuroendocrine carcinomas are locally advanced or metastatic at presentation. First-line systemic chemotherapy with a platinum agent (cisplatin or carboplatin) and etoposide is recommended for most patients with metastatic-stage disease; however, response durations are often short. Sequential or concurrent chemoradiation is recommended for patients with loco-regional disease. In patients with localized tumors undergoing surgical resection, adjuvant treatment (chemotherapy with or without radiation) is warranted in most cases.

Carcinoids are tumors derived from neuroendocrine cells and often produce functional peptide hormones. Approximately 54.5% arise in the gastrointestinal tract and frequently metastasize to the liver. Primary hepatic carcinoid tumors (PHCT) are extremely rare; only 95 cases have been reported. A 65-year-old man came to our attention due to occasional ultrasound findings in absence of clinical manifestations. His previous medical history, since 2003, included an echotomography of the dishomogeneous parenchymal area but no focal lesions. A computed tomography scan performed in 2005 showed an enhanced pseudonodular-like lesion of about 2 cm. Cholangio-magnetic resonance imaging identified the lesion as a possible cholangiocarcinoma. No positive findings were obtained with positron emission tomography. Histology suggested a secondary localization in the liver caused by a low-grade malignant neuroendocrine tumor. Immunohistochemistry was positive for anti chromogranin antibodies, Ki67 antibodies and synaptophysin. Octreoscan scintigraphy indicated intense activity in the lesion. Endoscopic investigations were performed to exclude the presence of extrahepatic neoplasms. Diagnosis of PHCT was established. The patient underwent left hepatectomy, followed by hormone therapy with sandostatine LAR. Two months after surgery he had a lymph nodal relapse along the celiac trunk and caudate lobe, which was histologically confirmed. The postoperative clinical course was uneventful, with a negative follow-up for hematochemical, clinical and radiological investigations at 18 mo post-surgery. Diagnosis of PHCT is based principally on the histopathological confirmation of a carcinoid tumor and the exclusion of a non-hepatic primary tumor. Surgical resection is the recommended primary treatment for PHCT. Recurrence rate and survival rate in patients treated with resection were 18% and 74%, respectively.

Metastatic carcinoid tumors of the appendix are probably not as rare as is commonly thought. In five of thirteen cases diagnosed at the Stanford-Palo Alto Hospital, there was microscopic evidence of lymphatic invasion. Metastasis to regional nodes was demonstrated in two of the five. These two cases bring the total number reported in the medical literature to 31. One of the patients was a 14-year-old girl. The youngest patient previously cited with metastasis from carcinoid tumor of the appendix was 16 years of age.

We believe that all carcinoid tumors, regardless of location, are malignant lesions and urge careful pathologic study for evidence of lymphatic invasion. When such invasion is present, removal of regional nodes by right hemicolectomy seems indicated.

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Background

Colorectal carcinoids are often described as low‐grade malignant. However, no study has compared the survival between patients with colorectal carcinoids and those with carcinomas, in a large series. In addition, no global consensus has been established on the crucial determinants of metastasis in colorectal carcinoids.

Aim

To determine the predictive factors for metastasis in colorectal carcinoids and clarify their prognosis compared with adenocarcinomas.

Methods

Data of all patients diagnosed as having colorectal carcinoids were extracted from a large nationwide database of colorectal tumours, the Multi‐Institutional Registry of Large‐Bowel Cancer in Japan, for the period from 1984 to 1998. Risk factors for lymph node (LN) metastases and distant metastases were analysed among those who were undergoing surgery, by univariate and multivariate analysis. Cancer‐specific survival was also compared between patients with colorectal carcinoids and those with adenocarcinomas registered in the same period.

Results

Among the 90 057 cases of colorectal tumours that were diagnosed, a total of 345 cases of carcinoids were identified, including 247 colorectal carcinoids of those undergoing surgery. Risk factors for LN metastasis were tumour size ⩾11 mm and lymphatic invasion, whereas those for distant metastasis were tumour size ⩾21 mm and venous invasion. Colorectal carcinoids without these risk factors exhibited no LN metastasis or distant metastasis. Cancer‐specific survival of patients with colorectal carcinoids without metastasis was better than that of those with adenocarcinomas. However, the survival was similar between carcinoids and adenocarcinomas if the tumours had LN metastasis or distant metastasis.

Conclusions

The presence of metastasis in colorectal carcinoids could lead to survival that is as poor as in adenocarcinomas. Tumours ⩽10 mm and without lymphatic invasion could be curatively treated by local resection, but others would need radical LN dissection.

Carcinoid tumors are low-grade malignant tumors that arise from neuroendocrine cells. Primary renal carcinoid is extremely rare. We present a case of 57-year-old male with primary renal carcinoid tumor. Presently, the patient is on regular follow up and is doing well.

There are four types of gastric carcinoid tumors, classified according to their histology and malignant potential. Only a few cases of carcinoid tumors in patients infected with Helicobacter pylori (H. pylori) have been reported so far. We report a patient infected with H. pylori presenting with a small solitary gastric carcinoid tumor with very low proliferative rate and normal gastrin levels. The tumor was endoscopically removed and the patient received an eradication therapy against H. pylori. No signs of metastatic disease have been found so far during more than 3 year of follow-up. Infection with H. pylori may cause chronic gastritis with normal or elevated gastrin levels, leading to the development of gastric carcinoids by mechanisms unrelated to gastrin. Enterochromaffin-like cell tumors related to a chronic H. pylori infection may be considered as a distinct type of gastric carcinoid tumors.

Carcinoid tumors are neuroendocrine neoplasms derived from enterochromaffin cells. Skeletal metastases from carcinoid tumors are considered to be extremely rare. In this study, we present two cases of carcinoid tumors that metastasized to the bone. Furthermore, we review 50 published case reports and reveal the features of skeletal metastasis of carcinoid tumors. The first case involved a 59-year-old man with a history of multiple metastases of a lung carcinoid tumor. The patient complained of back pain and numbness in the lower limbs. Magnetic resonance imaging revealed metastases in the thoracic spine. A spinal decompression was performed and the symptoms were resolved. The second case involved a 74-year-old man had been diagnosed with a lung carcinoid tumor 15 years previously and complained of left thigh pain. A radiograph showed osteolytic lesions in the shaft of the left femur. We repaired the femur using an intramedullary nail following curettage of the tumor. A radiograph of the femur revealed a callus on the pathological fracture. The patient was able to walk using a crutch 3 months after surgery. We reviewed 50 cases that described skeletal metastasis from carcinoid tumors. The average age of the patients was 54.9 years and 33 patients (66%) were male. The most common site of skeletal metastasis was the spine. We also investigated the survival rate of patients who developed skeletal metastasis from carcinoid tumors. The findings showed that survival of patients who developed osteolytic skeletal metastasis was worse than that of patients who developed osteosclerotic skeletal metastasis.

Background

Lung carcinoid tumor and low grade glioma are two uncommon malignancies.

Patients and methods

We report the case of 24-year-old man who presented with respiratory disease. Imaging investigations showed a right lung tumor and histological analysis confirmed a typical carcinoid tumor. As part of initial staging, brain MRI revealed an asymptomatic right frontal lesion. First, a right pulmonary lobectomy was performed without adjuvant treatment. In second time, brain tumorectomy was performed. Histological examination confirmed the diagnosis of low grade glioma (LGG). The patient remained in complete remission 2.5 years after the initial diagnosis.

Results

This is the first case reporting the association between LGG and lung carcinoid tumor, while no association between LGG and a systemic tumor have been published to date. Association of lung carcinoid tumor with other malignant diseases has been reported but remained uncommon. Only minimal data support a potential molecular common origin.

Conclusion

This exceptional association may be fortuitous. However, their concomitant diagnoses suggest a potential association between both rare diseases. A genetic susceptibility remains possible.

Carcinoids are neuroendocrine tumours which may secrete hormones like gastrin, insulin, ACTH, etc. Liver is a common site for metastasis of carcinoid origin and an unusual site for a primary carcinoid tumour to arise.

We present the case of a 51-year-old Caucasian man with diarrhoea, weight loss, duodenum ulcers and a liver mass in ultrasonography. A primary hepatic carcinoid tumour with a Zollinger Ellison syndrome was diagnosed. Surgery resection was performed and the patient remained free of symptoms two years after, with normalisation of gastrin levels.

Primary hepatic carcinoid tumour represents an uncommon diagnosis, based on radiological and pathological features. The exclusion of different primary locations is necessary. Once associated with a Zollinger Ellison syndrome, diagnose may be more complicated and challenging since only 7 cases of hepatic carcinoids with gastrin secretion were reported in medical literature.

A review of medical literature is performed and diagnoses tools that should be used for an accurate diagnosis and available treatment approaches are commented here.

Background

Primary carcinoid tumors of the liver are uncommon and rarely symptomatic. The diagnosis of primary hepatic etiology requires rigorous workup and continued surveillance to exclude a missed primary.

Case Presentation

We present a case of a 62-year-old female with a primary hepatic carcinoid tumor successfully resected, now with three years of disease-free follow-up. We present a review of the current literature regarding the diagnosis, pathology, management, and natural history of this disease entity.

Conclusion

Primary carcinoid tumors of the liver are rare, therefore classifying their nature as primary hepatic in nature requires extensive workup and prolonged follow-up. All neuroendocrine tumors have an inherent malignant potential that must be recognized. Management remains surgical resection, with several alternative options available for non-resectable tumors and severe symptoms. The risk of recurrence of primary hepatic carcinoid tumors after resection remains unknown.

Metastasis to the breast is a rare occurrence, constituting fewer than 2% of all breast tumours. Of all metastatic tumours in the breast, most arise from contralateral breast primaries. Other reported primary solid tumour sites include melanoma; lung, gastric, and renal cancers; and approximately 29 cases of carcinoid tumour.

Ambiguous presentations and an absence of carcinoid syndrome features make accurate radiographic and histologic assessment of breast carcinoids challenging. Here, we report the case of a 52-year-old woman who presented with a mammographic abnormality in the left breast. Excisional biopsy revealed histopathology consistent with carcinoid. After an exhaustive work-up, carcinoid within the terminal ileum was ultimately identified, and the woman was diagnosed with metastatic breast carcinoid, an exceedingly rare entity. This paper describes the common mammographic, cytologic, and immunohistochemical features typical of metastatic breast carcinoid tumours, together with their common clinical features, prognosis, and treatment options.

Cardiac tumors are rare and usually indicate metastatic disease. Characterizing a tumor and reaching an exact diagnosis can be difficult. Diagnosis has been aided greatly by advances in imaging, such as cardiovascular magnetic resonance with the use of gadolinium-pentetic acid. Carcinoid tumors are neuroendocrine neoplasms that are found most often in the intestinal tract, although they can also develop in the lung, stomach, or heart. Herein, we report the case of a 72-year-old woman with a history of intestinal carcinoid disease and presenting symptoms of dizziness, fatigue, and chest pain. We used cardiovascular magnetic resonance with gadolinium enhancement to identify a large mass obstructing left ventricular outflow. The histopathologic results of an endomyocardial biopsy confirmed that the mass was a left-sided metastatic carcinoid cardiac tumor. To our knowledge, we are reporting the 1st combined use of clinical evaluation, cardiovascular magnetic resonance, and histopathologic studies to reach such a diagnosis.

The heart is an exceedingly rare site of metastatic involvement in carcinoid tumors. Only nineteen cases have been described in the literature over the past 30 years. We report here on a patient who presented with progressive carcinoid syndrome despite surgical resection of her liver metastases. She was found to have cardiac metastases on inidium-111-pentetreotide scintigraphy and subsequently underwent external beam radiation to the heart resulting in symptomatic palliation of her syndrome and objective radiographic response. To our knowledge, this is the first reported case of metastatic cardiac carcinoid treated with external beam irradiation.

INTRODUCTION

We describe a previously healthy 40-year-old woman with Cushing’s syndrome caused by adrenocorticotropic hormone (ACTH) secretion from metastatic carcinoid.

CASE REPORT

Over a 2-year period, the patient had multiple hospitalizations for uncontrolled hypertension, hyperglycemia, and hypokalemia. She had transient flushing, rashes, and a rapid weight gain. In addition, she developed anasarca and had a nontraumatic hip fracture 1 month before presentation. Subsequently, a hypertensive crisis resulted in admission to the intensive care unit and fine-needle aspiration of a liver lesion.

DISCUSSION

A diagnosis of metastatic carcinoid was established. She was transferred to our hospital for further evaluation and management. On arrival, she had the signs of Cushing’s syndrome. Despite extensive evaluation, her primary carcinoid tumor was not localized. She was treated successfully with bilateral adrenalectomy and octreotide.

CONCLUSION

This case illustrates how early recognition of the signs and symptoms of excess ACTH is important for prompt and appropriate treatment.