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Primary Testicular Carcinoid Tumor presenting as Carcinoid Heart Disease.

Chikkaraddi ML, Krishna S, Shetty M - Indian J Urol (2015 Jan-Mar)

Bottom Line: We report a hereto unreported instance, where a patient with a long-standing testicular mass presented with carcinoid heart disease, an uncommon form of carcinoid syndrome.He presented with symptoms of right heart failure, episodic facial flushing and was found to have severe right-sided valvular heart disease.His urinary 5-hydroxy indole acetic acid level was elevated.

View Article: PubMed Central - PubMed

Affiliation: Department of Urology, Father Muller Medical College, Mangalore, Karnataka, India.

ABSTRACT
Primary carcinoid tumors of the testis are very rare, and they seldom present with carcinoid syndrome. We report a hereto unreported instance, where a patient with a long-standing testicular mass presented with carcinoid heart disease, an uncommon form of carcinoid syndrome. He presented with symptoms of right heart failure, episodic facial flushing and was found to have severe right-sided valvular heart disease. His urinary 5-hydroxy indole acetic acid level was elevated. He underwent orchidectomy and the histopathology confirmed a testicular carcinoid tumor.

No MeSH data available.


Related in: MedlinePlus

Macroscopy: coronal section of the testis with spermatic cord. Inset: Enlarged testis. (b) Microscopy: monomorphic cells in the nested trabecular patter
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Figure 2: Macroscopy: coronal section of the testis with spermatic cord. Inset: Enlarged testis. (b) Microscopy: monomorphic cells in the nested trabecular patter

Mentions: A 49-year old male presented with history of pedal edema and breathlessness of 2 months duration. There was history of facial flushing, but no history of wheezing, diarrhea or abnormal blood pressure changes. There was no weight loss or abdominal symptoms. He did not have significant comorbid illnesses except for a long-standing right testicular swelling of 10 years duration. On examination, he had an early ejection systolic murmur in the pulmonary area and diastolic murmur in the tricuspid area. He had hepatomegaly secondary to the severe right heart failure. He had an enlarged, firm and nontender right testis while the left testis was normal. Lymph nodes including the supraclavicular lymph nodes were not palpable. His chest X-ray showed cardiomegaly [Figure 1a] with clear lung fields. Electrocardiogram showed right bundle branch block. Echocardiography revealed the presence of severe right heart disease in the form of dilated right heart chambers, dysplastic tricuspid, and pulmonary valves, with stenosis and regurgitation in both valves. Hence, we evaluated him for carcinoid syndrome. His 24-h urine 5-hydroxy indoleacetic acid (5-HIAA) was elevated, whereas other serum markers for testicular tumors were normal [Table 1]. Computed tomography of the abdomen showed hepatomegaly and infrarenal para-aortic lymphadenopathy, but there were no intestinal tumors. The right testis was enlarged (4.8 × 5.0 × 7.6 cm), vascular, showing heterogeneous density, necrosis, and calcifications [Figure 1b] along with minimal hydrocele. Combined together, these findings favored the diagnosis of a testicular carcinoid tumor. Under local cord block, sedationand continuous cardiac monitoring, he underwent right radical high inguinal orchidectomy [Figure 2]. The right testis measured 3 × 4.5 × 8 cms, with the cut section showing a well-circumscribed tumor with pale white to pale yellow surface and with areas of hemorrhage and necrosis. Histological analysis showed a well-circumscribed encapsulated tumor composed of cells arranged in an acini, insular, and trabecular pattern [Figure 2]. The cells were round to oval with round nuclei, granular chromatin, and eosinophilic granular cytoplasm with collagenous stroma. Immunohistochemistry staining for synaptophysin expression was positive. All these features confirmed a carcinoid tumor. We made a diagnosis of a primary testicular carcinoid with carcinoid heart syndrome. He was advised repeat biochemical evaluation during follow-up visits, but he lost to follow up.


Primary Testicular Carcinoid Tumor presenting as Carcinoid Heart Disease.

Chikkaraddi ML, Krishna S, Shetty M - Indian J Urol (2015 Jan-Mar)

Macroscopy: coronal section of the testis with spermatic cord. Inset: Enlarged testis. (b) Microscopy: monomorphic cells in the nested trabecular patter
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4300575&req=5

Figure 2: Macroscopy: coronal section of the testis with spermatic cord. Inset: Enlarged testis. (b) Microscopy: monomorphic cells in the nested trabecular patter
Mentions: A 49-year old male presented with history of pedal edema and breathlessness of 2 months duration. There was history of facial flushing, but no history of wheezing, diarrhea or abnormal blood pressure changes. There was no weight loss or abdominal symptoms. He did not have significant comorbid illnesses except for a long-standing right testicular swelling of 10 years duration. On examination, he had an early ejection systolic murmur in the pulmonary area and diastolic murmur in the tricuspid area. He had hepatomegaly secondary to the severe right heart failure. He had an enlarged, firm and nontender right testis while the left testis was normal. Lymph nodes including the supraclavicular lymph nodes were not palpable. His chest X-ray showed cardiomegaly [Figure 1a] with clear lung fields. Electrocardiogram showed right bundle branch block. Echocardiography revealed the presence of severe right heart disease in the form of dilated right heart chambers, dysplastic tricuspid, and pulmonary valves, with stenosis and regurgitation in both valves. Hence, we evaluated him for carcinoid syndrome. His 24-h urine 5-hydroxy indoleacetic acid (5-HIAA) was elevated, whereas other serum markers for testicular tumors were normal [Table 1]. Computed tomography of the abdomen showed hepatomegaly and infrarenal para-aortic lymphadenopathy, but there were no intestinal tumors. The right testis was enlarged (4.8 × 5.0 × 7.6 cm), vascular, showing heterogeneous density, necrosis, and calcifications [Figure 1b] along with minimal hydrocele. Combined together, these findings favored the diagnosis of a testicular carcinoid tumor. Under local cord block, sedationand continuous cardiac monitoring, he underwent right radical high inguinal orchidectomy [Figure 2]. The right testis measured 3 × 4.5 × 8 cms, with the cut section showing a well-circumscribed tumor with pale white to pale yellow surface and with areas of hemorrhage and necrosis. Histological analysis showed a well-circumscribed encapsulated tumor composed of cells arranged in an acini, insular, and trabecular pattern [Figure 2]. The cells were round to oval with round nuclei, granular chromatin, and eosinophilic granular cytoplasm with collagenous stroma. Immunohistochemistry staining for synaptophysin expression was positive. All these features confirmed a carcinoid tumor. We made a diagnosis of a primary testicular carcinoid with carcinoid heart syndrome. He was advised repeat biochemical evaluation during follow-up visits, but he lost to follow up.

Bottom Line: We report a hereto unreported instance, where a patient with a long-standing testicular mass presented with carcinoid heart disease, an uncommon form of carcinoid syndrome.He presented with symptoms of right heart failure, episodic facial flushing and was found to have severe right-sided valvular heart disease.His urinary 5-hydroxy indole acetic acid level was elevated.

View Article: PubMed Central - PubMed

Affiliation: Department of Urology, Father Muller Medical College, Mangalore, Karnataka, India.

ABSTRACT
Primary carcinoid tumors of the testis are very rare, and they seldom present with carcinoid syndrome. We report a hereto unreported instance, where a patient with a long-standing testicular mass presented with carcinoid heart disease, an uncommon form of carcinoid syndrome. He presented with symptoms of right heart failure, episodic facial flushing and was found to have severe right-sided valvular heart disease. His urinary 5-hydroxy indole acetic acid level was elevated. He underwent orchidectomy and the histopathology confirmed a testicular carcinoid tumor.

No MeSH data available.


Related in: MedlinePlus