Cases reported "Pituitary Neoplasms"

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1/9. A novel germline mutation of multiple endocrine neoplasia type 1 (MEN1) gene in a Japanese MEN1 patient and her daughter.

    Familial multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant inherited disorder characterized by tumors of the parathyroid, anterior pituitary and gastro-entero-pancreatic endocrine tissues. The MEN1 gene has recently been cloned and its germline mutations have been considered to play an important role in the tumorigenesis of MEN1. We analyzed a Japanese MEN1 patient and her daughter for germline mutations of the MEN1 gene. The proband (60 y.o.) had primary hyperparathyroidism (PHP) and gastrinoma, and her daughter (30 y.o.) had prolactinoma. Clinical examinations revealed no evidence of PHP in the daughter. We identified a novel heterozygous germline mutation (712 A del) at codon 201 in exon 3 of the MEN1 gene in the proband. Restriction digestion analysis revealed the same mutation pattern in her daughter. These findings suggest that this family has familial MEN1 including a rare case of MEN1 with a single lesion of the pituitary. Genetic examinations are useful as diagnostic tools for any rare or variant case of familial MEN1.
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ranking = 1
keywords = tumorigenesis
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2/9. Pharyngeal pituitary: development, malformation, and tumorigenesis.

    The development of the pharyngeal pituitary (PhP) in the fetal period was morphologically and, for the first time, immunohistochemically examined. PhP, found in every individual, begins its hormone production at the 17-18th week of gestation, that is, 4-8 weeks later than that of sellar pituitary (SP). Only 1 of 25 examined fetuses without any stigmata of developmental anomalies showed a residual pituitary fragment in the craniopharyngeal canal (craniopharyngeal pituitary, CPhP). An adult case of a rare clivus pituitary adenoma that we examined is demonstrated in discussing its relationship to PhP. Extracranial ectopic pituitary adenomas in the literature describe an exclusively sphenoid sinus/nasopharyngeal/clivus location of the tumor. Their location corresponded exactly with that of PhP, so that the origin of the tumors can be reasonably speculated as PhP, although another origin, e.g., CPhP, can not be excluded. A variety of malformations of PhP, although very rare, have been described for the fist time during the systemic examination of 16 fetuses with different cranioneural malformations, such as agenesis, unseparated PhP from SP (pharyngosellar pituitary), fragmentation, and residual pituitary tissue in the open craniopharyngeal canal. However, developmental anomaly of PhP was not specifically associated with cranioneural malformations except in cases of chromosomal aberrations. The hormone production in PhP in malformation cases tended to be retarded. Absence of SP was recorded in 50% of anencephalics in the literature; however, PhP was identified in all anencephalics in our series, independent of the existence of SP. This supports the opinion that agenesis of SP in anencephalics seems to be false information.
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ranking = 4
keywords = tumorigenesis
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3/9. danazol and multiple hepatic adenomas: peculiar clinical findings in an acromegalic patient.

    Hepatic adenomas are rare tumors; they have been associated with the use of anabolic steroids. acromegaly increases the overall risk of neoplasms. We report the case of a female acromegalic patient in whom multiple hepatic adenomas appeared soon after danazol treatment for uterine fibromatosis. Treatment of acromegaly with octreotide, followed by successful selective pituitary adenomectomy, stabilized the number and the size of the liver tumors. We suggest that oversecretions of GH and IGF-I were strong promoting factors for liver tumorigenesis in this patient.
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ranking = 1
keywords = tumorigenesis
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4/9. Pituitary macroadenoma in a 5-year-old: an early expression of multiple endocrine neoplasia type 1.

    multiple endocrine neoplasia type 1 (men 1) is associated with parathyroid, enteropancreatic, pituitary, and other tumors. The MEN1 gene, a tumor suppressor, is located on chromosome 11. Affected individuals inherit a mutated MEN1 allele, and tumorigenesis in specific tissues follows inactivation of the remaining MEN1 allele. men 1-associated endocrine tumors usually become clinically evident in late adolescence or young adulthood, as high levels of PTH, gastrin, or PRL. Because each of these tumors can usually be controlled with medications and/or surgery, men 1 has been regarded mainly as a treatable endocrinopathy of adults. Unlike in men 2, early testing of children in men 1 families is not recommended. We report a 2.3-cm pituitary macroadenoma in a 5-yr-old boy with familial men 1. He presented with growth acceleration, acromegaloid features, and hyperprolactinemia. We tested systematically to see whether his pituitary tumor had causes similar to or different from a typical men 1 tumor. Germ line dna of the propositus and his affected relatives revealed a heterozygous point mutation in the MEN1 gene, which leads to a His139Asp (H139D) amino acid substitution. The patient had no other detectable germ-line mutations on either MEN1 allele. dna sequencing and fluorescent in situ hybridization with a MEN1 genomic dna sequence probe each demonstrated one copy of the MEN1 gene to be deleted in the pituitary tumor and not in normal dna, proving MEN1 "second hit" as a tumor cause. Gsalpha mutation, common in nonhereditary GH-producing tumors, was not detected in this tumor. We conclude that this pituitary macroadenoma showed molecular genetic features of a typical men 1-associated tumor. This patient represents the earliest presentation of any morbid endocrine tumor in men 1. A better understanding of early onset men 1 disease is needed to formulate recommendations for early men 1 genetic testing.
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ranking = 1
keywords = tumorigenesis
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5/9. hyperthyroidism in a patient with TSH-producing pituitary adenoma coexisting with thyroid papillary adenocarcinoma.

    A 27-year-old woman who presented with a left thyroid nodule was found to have hyperthyroidism caused by a syndrome of inappropriate secretion of TSH. The levels of free T3, free T4 and TSH were 9.50 pg/mL, 4.05 ng/dL and 2.16 microU/mL, respectively. magnetic resonance imaging of the head revealed a pituitary macroadenoma. The TSH response to TRH stimulation was normal and responses of other anterior pituitary hormones to stimulation tests were also normally preserved. Administration of octreotide with iodine successfully reversed hyperthyroidism prior to total resection of pituitary adenoma, which was followed by hemithyroidectomy of the left thyroid five months later. Histologically, the resected pituitary adenoma was a TSH-producing adenoma (TSH-oma) and the thyroid nodule was a papillary adenocarcinoma. serum TSH diminished to undetectable levels immediately following pituitary adenomectomy but gradually normalized over nine months. Coexistence of a TSH-oma with thyroid cancer is very rare and only two similar cases have previously been documented. This combination raises the possibility that TSH may be involved in tumorigenesis in the thyroid gland.
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ranking = 1
keywords = tumorigenesis
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6/9. Distinct clonal composition of primary and metastatic adrencorticotrophic hormone-producing pituitary carcinoma.

    The pathogenetic mechanisms underlying pituitary tumorigenesis are largely unknown. Previous reports have suggested that aggressive pituitary adenomas and/or carcinomas may be associated with genetic alterations that are distinct from those responsible for the more common and less aggressive pituitary adenomas. Here, we describe the clonal composition of a pituitary carcinoma, its recurrence and its metastasis. The samples studied were from a 48-year-old woman who presented with recurrent Cushing's syndrome. During the 8-year course of her disease, she had an ACTH-producing pituitary carcinoma requiring two transsphenoidal procedures and resection of a metastatic cervical lymph node. Her disease remained active despite surgical resection, external beam irradiation and medical treatment with ketoconazole. Ultimately, bilateral adrenalectomy was performed to control the hypercortisolism. Morphological and immunohistochemical studies revealed that the primary and recurrent pituitary tumours and the metastatic lesion were an endocrine tumour with ACTH and growth hormone immunoreactivity. Primary, recurrent and metastatic tumour DNAs were analysed for X-chromosome inactivation and loss of heterozygosity (LOH) at several microsatellite loci on chromosomes 9,10, 11, 13 and 22. All three lesions were monoclonal in composition as suggested by the pattern of x chromosome inactivation of the PGK-1 allele. Moreover, the primary, recurrent and metastatic lesions demonstrated LOH at the microsatellite allelic markers PYGM and D10S217. In contrast, however, the metastatic lesion showed a loss-to-retention pattern at two distinct loci (IFNA and D22S156) compared to the primary and recurrent pituitary tumours. These findings, while consistent with a clonal composition of the primary and metastatic pituitary lesions, show each clone to be distinct. This is the first description of a metastatic pituitary carcinoma with a distinct clonal composition from its primary source.
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ranking = 1
keywords = tumorigenesis
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7/9. Gliomatosis cerebri and pituitary adenoma: case report and literature review.

    The diagnosis of Gliomatosis cerebri (GC) is known to be difficult and is still a matter of debate. We describe an in vivo case of GC associated with a pituitary tumor. A 47-year-old woman presented with short-term memory loss. A MRI revealed the presence of a pituitary enhancing tumor and a diffuse lesion involving the brain. A left pterional craniotomy with partial temporal lobectomy and removal of the pituitary lesion were performed in order to obtain diagnosis. The histological analyses showed a pituitary non-functioning tumor and a GC consisting of neoplastic oligodendrocytes and astrocytes. Both lesions showed nuclear immunoreactivity for progesterone receptors (PGr) and estrogen receptors (EGr). This result could suggest there is a common receptor substrate in these tumors. In this case hormones could constitute a common step in tumorigenesis of both lesions.
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ranking = 1
keywords = tumorigenesis
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8/9. breast cancer in a male patient with prolactinoma.

    A 68-year-old man was diagnosed as having left primary breast cancer. Systemic bone roentgenography showed no evident metastasis, however, skull roentgenography revealed ballooning of the sella turcica, suggesting a pituitary tumor, which was subsequently confirmed by computed tomography. Because there was a high serum prolactin level, the pituitary tumor was diagnosed as a prolactinoma. A modified radical mastectomy was performed for the breast cancer, and bromocriptine therapy given for the prolactinoma. Prolactin is known to initiate and promote breast cancer in mice and rats but little is known about its role in human breast cancer. If hyperprolactinemia plays an important role in the tumorigenesis of human breast cancer as it does in mice and rats, the incidence of breast cancer in patients with hyperprolactinemia may be high. To our knowledge, however, only four such cases have been reported. The present rare case of male breast cancer with prolactinoma is discussed with reference to the literature.
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ranking = 1
keywords = tumorigenesis
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9/9. A rare case of gastric cancer in an acromegalic patient.

    Although it has been demonstrated that acromegalic patients have an increased risk of neoplasms, especially colon neoplasms, gastric cancer with acromegaly is very rare--only five cases have been reported to date in japan. We report here a rare case of gastric cancer with acromegaly in a 58-year-old woman, whose acromegalic change began at age 44. This patient showed typical clinical features of acromegaly, with increased concentrations of blood growth hormone (GH) and insulin-like growth factor i (IGF-I); she had four types of neoplasms; gastric cancer, colon tubular adenoma with moderate atypia, pancreatic mucinous cystadenoma, and subcutaneous lipoma. The gastric cancer was macroscopically 0-IIc type, 3.0 x 2.5 cm in size, and histologically diagnosed as a poorly differentiated adenocarcinoma with limited invasion of the mucosal layer. The previously documented stimulatory effects of GH and/or IGF-I on tumorigenesis and cell proliferation may have been responsible for the development of the multiple neoplasms in our patient.
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ranking = 1
keywords = tumorigenesis
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