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Over the past 15 years, I have reviewed the records of six patients whose physicians were alleged to have failed to diagnose a pituitary tumor before it bled ("apoplexy") or failed to recognize apoplexy after it had occurred. These cases were referred by attorneys involved in lawsuits alleging medical negligence. Their histories are summarized here from available clinical records, depositions, and other legal documents. None of these cases is still in litigation. The purpose of this commentary is to show how such legal case material is useful in alerting the medical community to pitfalls in diagnosis.
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PMID:Pituitary apoplexy goes to the bar: litigation for delayed diagnosis, deficient vision, and death. 1684 15

A series of 25 patients with a clinical diagnosis of pituitary apoplexy (PA) is reviewed. It included 14 men and 11 women aged between 20 to 79 years (mean age: 54 years). Twenty-two patients did not know that they had a pituitary tumor when the apoplexy occurred. A precipitating event was found in 3 cases. Symptoms and signs ranged from isolated ocular paresis to a deep coma. Seventeen patients experienced a decrease in their visual acuity. CTscan and MRI showed a pituitary adenoma in all cases, a hemorrhage was also present in 10 out of the 24 CTscans, and in all the 8 MRI performed. Twenty patients underwent surgery; 18 of them by a transsphenoidal approach. A complete recovery of visual acuity was observed in 75% of patients operated within the week following the onset of symptoms, and in 56% of patients operated later on. There was no case of complete visual recovery among the blind patients. Pituitary apoplexy is a clinical concept. It applies only to symptomatic cases. It is generally a complication of a pituitary adenoma which is in most cases unknown. There are different degrees of severity; PA can even be life-threatening. The principal aim of surgery in the acute phase is the improvement of visual prognosis. In our series, blind patients or those with a history of visual loss for more than a week or with a blindness had a poorer prognosis.
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PMID:[Pituitary apoplexy. Report of 25 patients]. 1708 13

LHRH analogs have become a promising modality in prostate cancer therapy as an alternative to surgical castration, and the use of these agents is generally considered to be safe. Since now, only few cases of an apoplexy of previously undiagnosed pituitary adenoma (usually gonadotropinoma) at the beginning of therapy have been described in the medical literature. We present a case of a 74 year old patient who was diagnosed of prostate cancer at the age of 68. There was no evidence of metastatic disease. Radical prostatectomy was performed and LHRH analog gosereline (Zoladex 3.6 mg s.c.) was administered. During the first day after gosereline injection the patient developed headaches that became more severe over the next 3 days. Then the patient experienced nausea and vomiting, double vision and eyelid ptosis. On the 5th day the patient temporarily lost consciousness and was admitted to hospital. Imaging (computerized tomography, magnetic resonance imaging) revealed the presence of a pituitary tumor and hemorrhage within the gland. There was no evidence of pituitary dysfunction in hormonal studies. Neurosurgical intervention was postponed for 5 days after admission. Pathological mass with signs of recent hemorrhage was removed via transsphenoidal route. The tumor had negative immunohistochemical GH, ACTH and PRL staining. Neurological impairment resolved within 9 months after the operation. As a result the patient required adrenal and thyroid replacement. During 6 years of follow-up there was no evidence of prostate cancer recurrence.
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PMID:Apoplexy of clinically silent pituitary adenoma during prostate cancer treatment with LHRH analog. 1715 26

The combination of pituitary gland tumor and aneurysmal disease has previously been described. Most of these aneurysms have affected intracranial arteries. The purpose is to present 2 patients with thoracoabdominal aortic aneurysm and pituitary gland tumor and further to discuss the mechanism behind this combination of diseases. A 59-year-old male patient was admitted with abdominal pain and a 120 mm thoracoabdominal aortic aneurysm type III. He was operated with resection and graft replacement. During the operation, it was noted that his intra-abdominal arteries were extremely enlarged. The diagnosis acromegaly was confirmed in the late 50's and he had received irradiation therapy and underwent partial trans-sphenoidal hypophysectomy. His growth hormone values eventually declined while he had elevated insulin growth factor-1 (IGF-1) levels. The patient died from stroke 6 years after operation. Patient n. 2 is a 73-year-old female with a type II thoracoabdominal aortic aneurysm. She was operated for a pituitary adenoma in 1988. There were no clinical or biochemical signs of acromegaly. However, she had elevated serum values of IGF-1. The maximum diameter of the aneurysm was 60 mm. Because of comorbidity the patient has been followed at the outpatient clinic. The mechanism behind the combination of pituitary gland tumor and aneurysm is obscure. One of our patients had classical acromegaly. Growth hormone decreased over the years, while his IGF-1 values were normal or elevated. The other patient had increased levels of IGF-1 without typical acromegaly. This might indicate that IGF-1 could play a role in the development of aneurysm in patients with pituitary tumor. This combination of diagnoses should be kept in mind when dealing with patients having aneurysmal disease.
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PMID:Is insulin growth factor-1 (IGF-1) playing a role for aneurysm formation in patients with pituitary gland tumor? 1716 53

Pituitary apoplexy is a clinical syndrome occurring as a consequence of fulminant expansion of pituitary tumor due to massive infarction, necrosis, and hemorrhage. Its association with head injury is rare and only few reports are available. Shear forces on stalk and arterial vasospasm have been proposed to be the possible reasons. The clinical picture is characterized by sudden onset headache, visual symptoms, multiple cranial nerves involvement, meningismus, altered mental status, and hormonal dysfunction. Transsphenoidal decompression is the standard treatment but suprasellar and widespread extension of hematoma may need intracranial approach. We are reporting a rare association of head injury with pituitary apoplexy, where endonasal surgery proved to be a simple useful approach to evacuate contiguous intra-cerebral hematoma with excision of apoplectic pituitary adenoma.
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PMID:Post traumatic pituitary apoplexy with contiguous intra cerebral hematoma operated through endonasal route--a case report. 1731 41

We present the case of a female patient who developed chiasmatic apoplexy and menstrual alterations. CT scanning showed a suprasellar hemorrhage. She underwent surgery with the presumptive diagnosis of pituitary tumor. At surgery, we find a brown-grayish lesion involving left optic nerve and chiasm. Cavernous angioma was diagnosed by histopathology. Cavernous angiomas constitute nearly 15% of all central nervous system vascular malformations. Location at the optic pathway is very rare, but must to be ruled out in the diagnosis of a patient with chiasmatic and/or optic apoplexy. Surgery is useful in preventing worsening of the previous deficit or a new visual defect.
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PMID:[Cavernous angioma of the second cranial nerve and chiasmatic apoplexy]. 1739 47

Rhinocerebral mucormycosis is an acute, fulminating form of invasive fungal sinusitis occurring principally in individuals who are immunologically or metabolically compromised. The incidence of pituitary apoplexy ranges from 6% to 17%, presenting as a capsule rupture in up to 1.7-2.0%. Isolated cases of mucormycosis are associated with solid tumors and Cushing's syndrome. A 42-year-old, diabetic woman, with Cushing's syndrome of 5 years duration presented with hemiplexy, hemiparesis and altered speech following a syncopal episode and fall. Brain CT scan showed a left temporal lobe infarction. The patient deteriorated rapidly and she died 4 days later. Autopsy findings included: plurihormonal pituitary adenoma with extension to the sphenoid bone and sellar erosion; many thick, septated, mucormycosis hyphae; and recent fronto-temporal brain infarction. Also, a solitary adrenal corticotropic hormone (ACTH)-producing neuroendocrine tumor, 3 cm in diameter, was found in the left lung. This patient illustrates the correlation between ACTH-producing ectopic pulmonary tumor, pituitary apoplexy and mucormycosis.
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PMID:Pituitary apoplexy due to mucormycosis infection in a patient with an ACTH producing pulmonary tumor. 1746 4

Concomitant thyroid cancer with pituitary tumor is uncommon. This study reports a case of advanced papillary thyroid carcinoma with pituitary adrenocorticotropic hormone (ACTH)-secreting tumor. A 58-year-old male patient had thyroid cancer in 1991 and presented with headache caused by pituitary tumor with apoplexy in 1993. Due to hypopituitarism, the patient underwent radioactive iodide ((131)I) for detection and treatment of metastatic thyroid cancer after the use of recombinant human thyroid-stimulating hormone (rhTSH) in 2000. During follow-up for thyroid cancer, (201)thallium scan proved to be an effective tool for detecting metastatic thyroid cancer in the patient without pituitary TSH reserve. Pituitary ACTH-secreting tumor was confirmed in 2001 based on the high serum ACTH level and positive immunohistochemical stain for ACTH. The patient had no Cushingoid features. Moreover, serum ACTH levels were 337 and 232 pg/mL with normal serum cortisol and urine-free cortisol. Although the patient underwent three operations and a total of 370 mCi (131)I therapy for recurrent thyroid cancer, the cancer continued to progress. Finally, the patient died of pneumonia with septic shock 12 years after the diagnosis of thyroid cancer.
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PMID:Concurrent papillary thyroid cancer with pituitary ACTH-secreting tumor. 1747 13

Aggressive pituitary tumors are rare the pathogenesis is not well established. The development of pituitary tumor after apoplexy has also been rarely reported. We describe the sequential development of Cushing's disease, apoplexy and aggressive pituitary tumor in the same patient. A 31-year old male presented with eutopic ACTH dependent Cushing's syndrome which failed initial pituitary surgery. He underwent subsequent bilateral adrenalectomy for control of hypercortisolism. An episode of pituitary apoplexy then occurred which was followed by the development of a null-cell pituitary tumor. This second tumor exhibited an aggressive behavior with invasion into the surrounding structures and systemic spread clinically. This case provides important evidence for the hypotheses of the pathogenesis of aggressive pituitary tumors which could have arisen from surviving adenoma cells following apoplexy or as a de novo development of pituitary carcinoma from cells which were not part of the original adenoma. This is the first report of a transformation of Cushing's disease to an aggressive and invasive null cell tumor after pituitary irradiation, apoplexy and surgery.
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PMID:Triple jeopardy in the pituitary. 1805 38

A 48-year-old female presented with a sudden onset of headache and visual impairment. Nineteen years before, she had undergone a transsphenoidal surgery for a prolactin producing pituitary adenoma at our hospital without intraoperative arterial bleeding. On arrival, she exhibited dilated pupils and loss of bilateral visual acuity, but improved immediately after all examinations. MRI revealed a pituitary tumor with intratumoral hemorrhage, intraventricular hemorrhage and subdural hemorrhage. Cerebral angiography revealed a left intracavernous carotid artery aneurysm. Her medical history and radiological findings suggested the rupture of a de novo aneurysm causing a hemorrhage into a pituitary adenoma mimicking pituitary apoplexy. Endovascular occlusion of the aneurysm was performed by use of platinum coils. Because of rapid improvement of visual acuity, administration of terguride was chosen for shrinking the pituitary adenoma. If a pituitary adenoma is present, the possibility of a coincidental aneurysm should always be considered. This association should be kept in mind when evaluating any case of pituitary apoplexy.
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PMID:[A case of ruptured internal carotid artery aneurysm mimicking pituitary apoplexy]. 1808 May 17


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