An unusual cause of tricuspid and pulmonary valve stenosis and regurgitation

Carcinoid heard disease is a frequent sequela to metastatic carcinoid tumour. This rare condition has a typical pathological presentation with characteristic macroscopic and microscopic findings. We present here a case detailing the classical findings of carcinoid heart disease in a tricuspid valve.     

Carcinoid tumour metastatic to the orbit with infiltration to the extraocular orbital muscle

Ninety-three percent of symptomatic patients with small intestinal carcinoid tumours have metastases. The most common sites of metastases are lymph nodes and liver. Orbital metastases have rarely been described and the majority of them involve the choroid rather than extraocular orbital structures. We report a patient who developed proptosis, impairment of vision and reduced ocular motility on the left side, eighteen months after operation for primary intestinal carcinoid tumour with hepatic metastases. CT and MR studies revealed the tumour mass infiltrating the inferior rectus muscle. Biopsy examined by imprint and frozen section showed tumour consistent with metastatic carcinoid. The tumour was removed. HE and staining for cytokeratin, chromogranin, NSE, serotonin, somatostatin and gastrin showed that the tumour tissue corresponded to that of the primary intestinal carcinoid tumour. Intramuscular orbital metastasis from a carcinoid tumour is a rare occurrence. Diagnosis may be difficult, especially where no evidence of primary carcinoid tumour is present. Metastatic orbital carcinoid should be suspected in patients with a clinical history of carcinoid tumour and who develop ocular complaints and mass lesion in the orbit. Complete surgical removal of the tumour is important for optimal restitution of vision and eye movements.     

Pure testicular carcinoid associated with intratubular germ cell neoplasia

This case report describes for the first time a case of pure testicular carcinoid pre-aortic lymph node metastases in a 25 year old patient with carcinoid syndrome. The simultaneous occurrence of intratubular germ cell neoplasia in the surrounding testicular tissue was identified by OCT4 and placental-like alkaline phosphatase positivity. This confirmed that the tumour had a germ cell origin in the testis, rather than being a metastasis from an extragenital carcinoid.     

CARDIOVASCULAR LESION OF CARCINOID SYNDROME An Autopsy Case of Bronchial Carcinoid

An autopsy case of a 67-year-old Japanese male is presented. He had been suffering from carcinoid syndrome for 5 years and showed a typical picture of carcinoid heart disease. In Japan, carcinoid heart disease is rare and we can find only four reported cases (33% of reported carcinoid syndrome). The patient had high urinary secretion of 5-HIAA and high serum serotonin, and finally he died of heart failure and bronchopneumonia. The primary site of this carcinoid tumor was of the bronchus of the right B10c, and it had large hepatic metastases. Electronmicroscopically, the tumor cells had secretory granules measuring 1500–3500 Å in diameter. Immunohistochemically, the tumor cells were markedly positive for human chorionic gonadotropin (hCG) and antidiuretic hormone (ADH) and positive for serotonin, in both the primary site and hepatic metastases. Characteristic fibrous plaques were detected in the right atrium, tricuspid valve, right ventricle, and left atrium. Electron-microscopically, the fibrous plaques consisted of smooth muscle cells and myofibroblasts surrounded by basement membrane-like material. The abundant matrix of the fibrous plaques contained acid mucopolysaccharide, microfibrils and collagen fibers. The same fibrous plaques were also found in hepatic veins. Furthermore, retroperitoneal fibrosis was present, which showed proliferation of myofibroblasts, fibroblasts and immature mesenchymal cells.     

Cardiovascular lesion of carcinoid syndrome. An autopsy case of bronchial carcinoid

An autopsy case of a 67-year-old Japanese male is presented. He had been suffering from carcinoid syndrome for 5 years and showed a typical picture of carcinoid heart disease. In Japan, carcinoid heart disease is rare and we can find only four reported cases (33% of reported carcinoid syndrome). The patient had high urinary secretion of 5-HIAA and high serum serotonin, and finally he died of heart failure and bronchopneumonia. The primary site of this carcinoid tumor was of the bronchus of the right B10c , and it had large hepatic metastases. Electronmicroscopically, the tumor cells had secretory granules measuring 1500-3500 A in diameter. Immunohistochemically, the tumor cells were markedly positive for human chorionic gonadotropin (hCG) and antidiuretic hormone (ADH) and positive for serotonin, in both the primary site and hepatic metastases. Characteristic fibrous plaques were detected in the right atrium, tricuspid valve, right ventricle, and left atrium. Electron-microscopically, the fibrous plaques consisted of smooth muscle cells and myofibroblasts surrounded by basement membrane-like material. The abundant matrix of the fibrous plaques contained acid mucopolysaccharide, microfibrils and collagen fibers. The same fibrous plaques were also found in hepatic veins. Furthermore, retroperitoneal fibrosis was present, which showed proliferation of myofibroblasts, fibroblasts and immature mesenchymal cells.     

Pigmented spindle cell carcinoid tumour of the thymus with ectopic adrenocorticotropic hormone secretion: report of a rare variant and differential diagnosis of mediastinal spindle cell neoplasms

AIMS: A variety of histological variants of thymic carcinoid tumour have been described. A rare case of pigmented spindle cell carcinoid tumour of the thymus is documented and compared with the reported cases of thymic pigmented carcinoid tumour in the literature, with a discussion of the differential diagnosis of spindle cell tumours of the mediastinum. METHODS AND RESULTS: A thymic tumour with ectopic adrenocorticotropic hormone (ACTH) secretion was resected from a 24-year-old man suffering from Cushing's syndrome. Histological, immunohistochemical, and ultrastructural studies revealed an ACTH-producing spindle cell carcinoid tumour harbouring pigmented melanocytes. Among four thymic pigmented carcinoid tumours reported before, only one was similar to the present case by being also an ACTH-secreting pigmented spindle cell thymic carcinoid tumour. The clinicopathological features of this tumour distinguish it from a spindle cell thymoma, spindle cell thymic carcinoma, and other mediastinal spindle cell tumours. CONCLUSIONS: This case illustrates an extremely rare variant of thymic carcinoid tumour exhibiting a spindle cell morphology and harbouring pigmented melanocytes. Awareness of this histological variant is important in the differential diagnosis of spindle cell tumours of the mediastinum.     

Tumour suppressor gene products, proliferation, and differentiation markers in lung neuroendocrine neoplasms.

Typical carcinoid, atypical carcinoid, and small cell lung cancer (SCLC) fall within the spectrum of neuroendocrine lung neoplasms. This paper investigates the immunohistochemical expression of the products of tumour suppressor genes p53 and retinoblastoma (RB), together with proliferation (PCNA and Ki67) and neuroendocrine differentiation markers, in 14 typical carcinoids, ten atypical carcinoids, four borderline atypical carcinoid/SCLC, and 11 SCLC. We demonstrated that the phosphoprotein p53 and RB product can be immunolocalized on routine histological material. p53 protein was absent in all typical and atypical carcinoids, while it was abnormally expressed in eight SCLC and one borderline case. RB product was detected in all typical carcinoids and in two atypical carcinoids, while it was consistently absent in the other cases. PCNA-labelled cells were less than 4 per cent in typical carcinoids, about 40 per cent in atypical carcinoids, and over 70 per cent in SCLC. PCNA labelling index discriminates between typical and atypical carcinoids. Neuroendocrine differentiation was evaluated by a semi-quantitative method: a mean score value was obtained, which was high in typical carcinoids, intermediate in atypical carcinoids, and low in SCLC. Our data was obtained, which was high in typical carcinoids, intermediate in atypical carcinoids, and low in SCLC. Our data show that the decrease in neuroendocrine features from typical carcinoid to SCLC is paralleled by an increase in proliferative activity and by an altered expression of tumour suppressor gene products. The above findings have diagnostic relevance.     

A very rare case of synchronous primary lung cancer lymph node metastasis

A 76 year old patient presented with two synchronous primary lung tumours. One was identified as an adenocarcinoma and the second as an atypical carcinoid tumour. When reviewing the lymph node slides it was seen that one lymph node contained metastatic deposits from both primary tumours. Synchronous lung tumours are fairly rare occurrences, but even rarer is the finding of synchronous metastases to the same lymph node. The atypical carcinoid deposit was very subtle in appearance and could easily have been overlooked. This case demonstrates a useful learning point to not miss these rare and subtle findings as the resulting tumour staging was affected and may have implications for further patient management.     

Bronchial neuroendocrine (carcinoid) tumor causing unilateral left-sided carcinoid heart disease

Summary A female patient suffering from a bronchial neuroendocrine tumor with unilateral leftsided carcinoid heart disease is reported. Repeated x-ray films of the chest showed a slowly growing lung tumor in the left lower lobe. The patient refused any diagnostic or therapeutic procedure to define the type of the tumor. During the follow-up of 24 years she developed severe mitral and moderate to severe aortic insufficiency, both invasively quantified by thermodilution techniques. During surgery for double valve replacement the patient died from left ventricular heart failure. Necropsy revealed the typical pattern of a bronchial neuroendocrine tumor without metastases. Examination of the heart disclosed the characteristic deposits of fibrous tissue on the cusps of both the mitral and the aortic valves whereas the right heart showed no abnormalities. Review of the literature suggests the unilateral left-sided carcinoid heart disease to be a very rare finding, its pathogenesis remains to be elucidated.     

Virilization due to ovarian androgen hypersecretion in a patient with ectopic adrenocorticotrophic hormone secretion caused by a carcinoid tumour: case report

A 52-year-old woman presented with symptoms of virilization, which had been ongoing for 5 months. At the age of 34 years, she had a large abdominal carcinoid tumour removed. Twelve years later, she presented with Cushing's syndrome due to ectopic adrenocorticotrophic hormone (ACTH) production by carcinoid metastases localized in the right parametrium, fornix posterior and right diaphragm. Debulking laparotomy was performed followed by remission of hypercortisolism. Relapse of hypercortisolism followed 3 years later, and a second debulking laparotomy was performed including resection of the right ovary. In the following year, relapses of hypercortisolism were observed until bilateral adrenalectomy was performed. Laboratory evaluation revealed elevated serum levels of testosterone (23.0 nmol/l), androstenedione and 17-hydroxyprogesterone, and a serum estradiol (E2) level in the premenopausal range. The computerized tomography (CT) of the abdomen showed a large pelvic mass on the left side of the uterus without a recognizable left ovary. Treatment with a GnRH agonist (goserelin, 3.6 mg s.c., monthly) was initiated, resulting in normalization of the androgen levels. One year later, obstruction of the right ureter occurred due to progression of the pelvic metastases, thus a third debulking laparotomy with resection of the pelvic metastases including the left ovary was performed. The microscopic examination of the removed pelvic mass showed malignant carcinoid tissue with focal remnants of atrophic ovarian tissue. Two years after surgery, serum androgen levels are undetectable. We hypothesize that the high levels of ACTH at the site of the left ovary have induced androgen hypersecretion by steroid-producing cells in the ovary of our patient.     

An 18-year history of a corticotropin-secreting spindle cell carcinoid in the lung

A unique case is reported demonstrating latency and slow growth of a spindle-cell carcinoid in the lung. Seventeen years after bilateral adrenalectomy, performed for Cushing's syndrome, a patient presented with a newly developed lung tumor. The tumor demonstrated spindle cell histology, neurosecretory granules typical for carcinoid, and a positive immunoreactivity for corticotropin. After the removal of the lung tumor, the high serum corticotropin level returned to normal.     

Embryonal rhabdomyosarcoma of the larynx in a 63 year-old man]

A case of embryonal rabromyosarcoma localized in the larynx in the region of the left vocal bands in an elderly man is described, the tumour represented a hardly discernable delimited greyish-pinkish node with 1 cm diametre. Microscopic investigation of the tumour revealed the structure typical of embryonal rabromyosarcoma. The patient died two days after laryngectomy of thromboembolism of the pulmonary artery. At autopsy no metastases were observed. The peculiar features of the case are an extremely rare localization of embryonal rabromyosarcoma and advanced age of the patient.     

Midgut carcinoid tumor with carcinoid heart disease. Its presence in the absence of hepatic metastases--a case report

A 56-year-old man with a midgut carcinoid tumor had extensive metastatic involvement of lymphatic spaces and carcinoid heart disease. Unlike similar previously reported cases with carcinoid heart disease, hepatic metastases and history of the carcinoid syndrome were absent in this patient. Tumor involvement of cardiac lymphatic spaces may have been responsible for the development of carcinoid heart disease due to a local fibrogenic effect similar to the extensive fibrosis associated with tumor seen within the abdominal cavity at autopsy.     

Carcinoid of the larynx

Laryngeal carcinoid with metastases to cervical lymph nodes was observed in a patient of 62. The diagnosis was confirmed by electron-microscopic study. The patient was followed up for 9 mos without any evidence of recurrence and metastases after laryngectomy and fascial envelope excision of cervical fat. The morphologic picture of laryngeal carcinoid is characterized by the presence of the alveolar, trabecular and teniform structures consisting of monomorphic and moderately polymorphic cells. Tubular and rosette-like structures occur. The overlying epithelium is intact. The necrotic foci, as a rule, are absent. Grimelius reaction is positive, argentaffinnic granularity isn't revealed. Electron-microscopic study demonstrates endocrine-type granules. The need for recognition of laryngeal carcinoid is explained by the fact that this tumour form has a more favourable course and prognosis in comparison to cancer.     

Adamantinoma of tibia: a case of late local recurrence along with lung metastases

Adamantinomas of long bones are rare primary low-grade malignant tumours composed of cells with epithelial and fibrous characteristics. Local recurrence, though scarce, occurs 5-15 years after the onset of diagnosis. We report a case of local recurrence of an adamantinoma localised in tibia, along with the presence of two lung metastases, 24 years after diagnosis and surgical therapy of the primary tumour. The local recurrence and the lung metastases were removed surgically. The patient remains free of the disease for 3 years.     

Liver-originating isoenzymes of alkaline phosphatase in the serum: a paraneoplastic manifestation of a malignant schwannoma of the sciatic nerve.

A case of malignant schwannoma of the sciatic nerve is described associated with hepatic dysfunction in the absence of hepatic metastases. An elevated serum alkaline phosphatase activity was present with an isoenzyme pattern indicating hepatic involvement. These abnormalities disappeared after extirpation of the tumour. The patient is well, with no evidence of metastases, over two years later. It is concluded that the abnormality of serum alkaline phosphatase was induced by the tumour, and that the liver can be involved in the paraneoplastic syndrome.     

Primary carcinoid tumor of the larynx and review of the literature

Primary carcinoid tumor of the larynx is very rare. This is the fifth case reported in the literature and the first autopsy case of laryngeal carcinoid. The early manifestation of the present case was multiple metastasis to the skin. At autopsy there was a laryngeal tumor associated with widespread visceral and cutaneous metastases. The tumor proved to be carcinoid tumor by histological, histochemical and electron microscopic findings.     

PRIMARY CARCINOID TUMOR OF THE LARYNX AND REVIEW OF THE LITERATURE

Primary carcinoid tumor of the larynx is very rare. This is the fifth case reported in the literature and the first autopsy case of laryngeal carcinoid. The early manifestation of the present case was multiple metastasis to the skin. At autopsy there was a laryngeal tumor associated with widespread visceral and cutaneous metastases. The tumor proved to be carcinoid tumor by histological, histochemical and electron microscopic findings.     

Malignant carcinoid of the pancreas: a cytologic, ultrastructural, and immunocytochemical study of a case diagnosed by fine-needle aspiration of a supraclavicular node metastasis

Carcinoid tumor of the pancreas is extremely rare. This article describes the case of a 22-year-old woman who manifested a typical carcinoid syndrome; a definitive diagnosis of a metastatic carcinoid tumor was made from needle aspiration of a supraclavicular lymph node using morphologic, immunocytochemical, and ultrastructural criteria. The carcinoid tumor was subsequently shown to be of pancreatic origin.     

Minute carcinoid arising in gastric tubular adenoma

We report a minute carcinoid tumour forming multiple, endocrine nodules or glandular structures and located within a gastric tubular adenoma. The tumour cells were positive for the argyrophilic reaction and a few revealed serotonin immunoreactivity. This case may represent the third histogenetic type of gastric carcinoid tumour.