Who was Dr. William C. Baum?

The first discovery of primary hyperaldosteronism secondary to an aldosterone-secreting adrenal adenoma has been credited solely to Dr. Jerome Conn, an endocrinologist at the University of Michigan and for whom, Conn syndrome was named. Dr. William Baum, a urologist at the University of Michigan, however, was instrumental in the appropriate operation and historical aldosteronoma resection. Despite Dr. Baum's important role in this discovery, he was never included as an author in any of the subsequent papers describing Conn syndrome and, few today would recognize his name. So, who was Dr. Baum and what happened? This historical article aims to revisit the history surrounding the discovery of aldosteronoma as a cause of Conn's syndrome and to catalog the life and involvement of Dr. William C. Baum in that discovery.

Parathyroid surgery: from inception to the modern day.

Parathyroid surgery has undergone great changes since its inception less than a century ago. It is still the only definitive option available to cure primary or tertiary hyperparathyroidism. This review details the development of parathyroid surgery, our understanding of hyperparathyroidism and the treatment options available. It also discusses the technological advances that have enabled parathyroid localization and prediction of surgical success.

Pituitary adenomas: historical perspective, surgical management and future directions.

Pituitary adenomas are among the most common central nervous system tumors. They represent a diverse group of neoplasms that may or may not secrete hormones based on their cell of origin. Epidemiologic studies have documented the incidence of pituitary adenomas within the general population to be as high as 16.7%. A growing body of work has helped to elucidate the pathogenesis of these tumors. Each subtype has been shown to demonstrate unique cellular changes potentially leading to tumorigenesis. Surgical advancements over several decades have included microsurgery and the employment of the endoscope for surgical resection. These advancements increase the likelihood of gross-total resection and have resulted in decreased patient morbidity.

Skull deformations in craniosynostosis and endocrine disorders: morphological and tomographic analysis of the skull from the crypt of the Silesian Piasts in Brzeg (16th-17th century), Poland.

RESULTS: of morphological and tomographic (CT) studies of the skull that was found in the crypt of the Silesian Piasts in the St. Jadwiga church in Brzeg (Silesia, Poland) are presented and discussed here. The established date of burial of probably a 20-30 years old male was 16th-17th century. The analyzed skull showed premature obliteration of the major skull sutures. It resulted in the braincase deformation, similar to the forms found in oxycephaly and microcephaly. Tomographic analysis revealed gross pathology. Signs of increased intracranial pressure, basilar invagination and hypoplasia of the occipital bone were observed. Those results suggested the occurrence of the very rare Arnold-Chiari syndrome. Lesions found in the sella turcica indicated the development of pituitary macroadenoma, which resulted in the occurrence of discreet features of acromegaly in the facial bones. The studied skull was characterized by a significantly smaller size of the neurocranium (horizontal circumference 471 mm, cranial capacity ∼ 1080 ml) and strongly expressed brachycephaly (cranial index=86.3), while its height remained within the range for non-deformed skulls. A narrow face, high eye-sockets and prognathism were also observed. Signs of alveolar process hypertrophy with rotation and displacement of the teeth were noted. The skull showed significant morphological differences compared to both normal and other pathological skulls such as those with pituitary gigantism, scaphocephaly and microcephaly.

The history of autologous fat graft use for prevention of cerebrospinal fluid rhinorrhea after transsphenoidal approaches.

Presented herein is a review of the history of fat graft use in preventing iatrogenic cerebrospinal fluid (CSF) rhinorrhea after transsphenoidal surgery. Since the first transsphenoidal surgeries were described in the early 1900s, the techniques of sellar packing to prevent CSF leak have evolved. Kanavel, Halstead, and Cushing used bismuth- or iodine-soaked gauze. Under Dandy's influence, fascia lata was the first autologous material to be used for the repair and prevention of CSF rhinorrhea. The use of autologous fat graft for this purpose has only been reported in recent decades. Montgomery was the first to use abdominal fat to obliterate the middle ear cavity in 1964, and Collins reported the first transsphenoidal application of fat graft in 1973. Other reports by Kirchner, Tindall, and Wilson followed.

Hermann Schloffer and the origin of transsphenoidal pituitary surgery.

A little over a century ago, in 1907, at the University of Innsbruck, Hermann Schloffer performed the first transsphenoidal surgery on a living patient harboring a pituitary adenoma. Schloffer used a superior nasal route via a transfacial lateral rhinotomy incision. This was perhaps his greatest academic contribution to neurosurgery. Despite the technological limitations of that time, Schloffer's operation was groundbreaking in that it laid the foundation for future development and refinement of transsphenoidal pituitary surgery, influencing prominent surgeons such as Oskar Hirsch and Harvey Cushing. Even after undergoing multiple modifications and a brief fall into obscurity, the transsphenoidal approach has endured through generations of surgeons and remains the preferred approach for lesions of the sella turcica to this day. Although Schloffer performed primarily abdominal surgery in his practice, his contributions to the transsphenoidal approach have had a lasting impact in the field of pituitary and skull base surgery. The authors review the life and career of Hermann Schloffer, the surgical details of his transsphenoidal operation, and the legacy that it has left on the field of pituitary surgery.

The history of pituitary surgery for Cushing disease.

Although he never performed a pituitary operation for the disease, Harvey Cushing was the first to describe and treat patients with Cushing disease (CD). Other surgeons at the time were reluctant to operate on the pituitary due to the normal sella on skull radiographs in CD and the unclear etiology of the disorder. To better define and understand factors influencing the history of pituitary surgery for CD, the authors analyzed historical texts related to CD biology, diagnosis, and treatment. Cushing's monograph on basophilic pituitary adenomas and cortisol excess appeared in 1932. One year later in 1933, Alfred Pattison performed the first successful pituitary operation for CD by implanting radon seeds in the sella. Resection of a pituitary adenoma for CD was attempted 1 month later in 1933 by Howard Naffziger, resulting in only transient improvement that corresponded to the lack of tumor in the resected tissue. Soon thereafter, Susman in 1935 and Costello in 1936 described pituitary basophilic adenomas at autopsy in patients without premorbid endocrinopathy. They concluded that the adrenal gland was the cause of CD, which resulted in a 3-decade abandonment of pituitary surgery for CD. Jules Hardy in 1963 used the operating microscope to perform the first selective removal of an adrenocorticotropic hormone (ACTH)-secreting microadenoma, which established a pituitary cause and defined the modern treatment of CD. Subsequent reports by Hardy, Laws, and Wilson resulted in widespread acceptance of pituitary surgery for CD. Initial reluctance to operate on the pituitary for CD was multifaceted and included general uncertainty surrounding the etiology of Cushing syndrome as well as a lack of early surgical success, both due to the small size of ACTH-secreting adenomas. Selective removal of ACTH-secreting adenomas identified the source of CD and ended the delay in acceptance of pituitary surgery for CD.

AIP mutation in pituitary adenomas in the 18th century and today.

Gigantism results when a growth hormone-secreting pituitary adenoma is present before epiphyseal fusion. In 1909, when Harvey Cushing examined the skeleton of an Irish patient who lived from 1761 to 1783, he noted an enlarged pituitary fossa. We extracted DNA from the patient's teeth and identified a germline mutation in the aryl hydrocarbon-interacting protein gene (AIP). Four contemporary Northern Irish families who presented with gigantism, acromegaly, or prolactinoma have the same mutation and haplotype associated with the mutated gene. Using coalescent theory, we infer that these persons share a common ancestor who lived about 57 to 66 generations earlier.

Early descriptions of acromegaly and gigantism and their historical evolution as clinical entities.

Giants have been a subject of fascination throughout history. Whereas descriptions of giants have existed in the lay literature for millennia, the first attempt at a medical description was published by Johannes Wier in 1567. However, it was Pierre Marie, in 1886, who established the term "acromegaly" for the first time and established a distinct clinical diagnosis with clear clinical descriptions in 2 patients with the characteristic presentation. Multiple autopsy findings revealed a consistent correlation between acromegaly and pituitary enlargement. In 1909, Harvey Cushing postulated a "hormone of growth" as the underlying pathophysiological trigger involved in pituitary hypersecretion in patients with acromegaly. This theory was supported by his observations of clinical remission in patients with acromegaly in whom he had performed hypophysectomy. In this paper, the authors present some of the early accounts of acromegaly and gigantism, and describe its historical evolution as a medical and surgical entity.

Hyalinizing trabecular tumors of the thyroid gland: quadruply described but not by the discoverer.

Hyalinizing trabecular tumors of the thyroid have been described on 4 occasions, by Carney and colleagues in 1987, by Ward and coworkers in 1982, by Pierre Masson in 1922, and by Rahel Zipkin in 1905. Zipkin credited her chief, Theodor Langhans (of Langhans giant cell fame), with identification of the cases she reported. Unaware of the 3 earlier descriptions, Carney and colleagues described 11 circumscribed or encapsulated thyroid tumors with elongated and polygonal cells arranged in trabeculae that contained a hyaline material resembling amyloid. The nuclei of the tumor cells had cytoplasmic invaginations and grooves similar to those of papillary carcinoma. Carney and colleagues labeled the neoplasms hyalinizing trabecular adenomas because of their microscopic appearance, absence of invasion, and benign natural history. Subsequently, the nuclear features of the tumor and the molecular genetic findings led to the introduction of equivocal designations for it, hyalinizing trabecular tumor and hyalinizing trabecular neoplasm, and later to its designation as a variant of papillary carcinoma. Experience has shown that most circumscribed or encapsulated follicular thyroid tumors with intratrabecular hyalin and nuclear features of papillary carcinoma behave as benign neoplasms. Hyalinizing trabecular carcinoma is a very rare tumor.

A century of pituitary surgery: Schloffer's legacy.

Although pituitary tumors were well recognized by the end of the 20th century, very few surgeons had attempted to remove such tumors. In 1906, Hermann Schloffer at the Innsbruck University Clinic of Surgery reviewed the subject of pituitary surgery. Very little was known about the function of the pituitary, and no reports had yet been published on attempts to surgically remove pituitary neoplasms. Schloffer pointed out that ophthalmological symptoms evidently reflected mass effect of the tumor and, hence, resection of the tumor would be beneficial. He also proposed a transsphenoidal approach to the sella, although he was well aware of the risk of meningitis associated with this procedure. On March 16, 1907, Schloffer performed the first transsphenoidal operation for a pituitary adenoma. Unfortunately, the patient died 2 months later from a large residual tumor. Within a few years, several patients had undergone operation with procedures similar to the one described by Schloffer, several of whom experienced acromegaly, thus introducing neurosurgery as routine therapy for acromegaly and settling the long debate on the etiology of acromegaly.

The history of the treatment of pituitary adenomas.

The immense history leading to our current understanding and treatment of pituitary pathology is inextricably linked to the evolution of the understanding of the numerous functions of the hypophysis cerebri as the "master gland" of the endocrine system. When the anatomists of old encountered this small organ sequestered "like a nugget in the innermost of Chinese boxes" at the base of the brain, they had no inkling of its importance in the control of multiple target organs in the human body. It would ultimately take two millennia and a vast amount of clinical and laboratory research for its role in the body to eventually become more completely appreciated.

Update on the medical management of pituitary adenomas.

The medical treatment of pituitary adenomas has changed significantly over the past decade. Pharmacologic therapy for prolactinomas in the form of dopamine agonists has been available since the 1970s, and somatostatin analogues for treatment of growth hormone (GH)-secreting adenomas were introduced in the 1980s. However, the recent introduction of long-acting forms of these agents has markedly improved efficacy. Furthermore, long-acting somatostatin analogues also have utility in treating thyrotropin adenomas and a subset of adrenocorticotroph tumors. Limited clinical studies with long-acting dopamine agonists suggest that a subset of patients with GH, adrenocorticotroph, and gonadotropin/nonsecreting adenomas may also benefit from therapy with these agents. The introduction of a GH receptor antagonist in the 1990s has added to the pharmacologic armamentarium for treatment of acromegaly. In parallel with improved medical therapy, hormonal assays for assessing tumor activity have improved in sensitivity, necessitating new standards for treatment optimization. This article highlights some of these evolving new ideas and approaches to the pharmacologic management of pituitary adenomas.