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Important to mention is the occurrence of basophilic invasion of the posterior lobe. Although this is a normal process without clinical significance, its recognition is important for preventing misinterpretation as a basophilic adenoma invading the posterior gland. This classification has helped not just to understand further the cytogenesis of pituitary adenomas but also to identify different tumor types that may present clinically with similar symptomatology. Pituitary adenomas are classified clinically into two main groups: the clinically functioning adenomas and the clinically nonfunctioning adenomas, according to whether or not an endocrine syndrome is present. These clinically nonfunctioning adenomas commonly present with symptoms related to local mass effect such as headaches; neurologic deficits in the cranial nerves, including visual field disturbances; and mild hyperprolactinemia resulting from pituitary stalk compression (the so-called stalk effect). According to tumor size and gross anatomic features, adenomas are divided into microadenomas (tumors <1 cm in diameter) and macroadenomas (tumors >1 cm in diameter). Macroadenomas show an increased tendency toward suprasellar extension, gross invasion, and recurrence. A radiologic classification, proposed by Hardy,16 is the most widely used in clinical practice. Morphologically, adenomas may show a variety of histologic growth patterns, including diffuse, papillary, and trabecular arrangements similar to other neuroendocrine tumors. Although these histologic features are of no prognostic significance, their recognition is important in the study of the various lesions that may be considered in the differential diagnosis of pituitary adenomas. Cytologically, tumor cells may be acidophilic, basophilic, or chromophobe; however, these tintorial characteristics do not identify specific adenoma types.

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The characteristic biphasic appearance in typical cases of infantile myofibromatosis rarely allows any differential diagnosis, although very cellular lesions may show morphologic overlap with infantile fibrosarcoma,176 sometimes requiring molecular testing for their distinction. The organoid growth pattern of these lesions remains unexplained but allows for no real differential diagnosis. Infantile myofibromatosis,170-172 formerly known as congenital generalized fibromatosis, typically presents before the age of 2 years, is congenital in up to 30% of cases, and shows a moderate preponderance in boys. At most 10% of patients have multiple lesions, although this figure was thought to be higher in the past. The majority of lesions arise in skin and superficial soft tissue, especially of the head and neck region or trunk, but lesions in bone are also quite common173 (see Chapter 25), and, in multicentric cases, very occasionally visceral involvement may be seen, especially of the gastrointestinal tract or lungs. A small proportion of cases are inherited, seemingly in an autosomal dominant fashion. Typically abrupt transition from myofibroblastic spindle cell area to more primitive rounded cells at the periphery. In this case the less differentiated areas consist of rounded cells with eosinophilic cytoplasm arranged around branching vessels. Juvenile Hyaline Fibromatosis Juvenile hyaline fibromatosis177,178 is an exceedingly rare disorder of infants and children that appears to have autosomal recessive inheritance. It is characterized by multiple slowly growing dermal or subcutaneous tumors, especially in the head and neck region and upper trunk, often associated with gingival hypertrophy, severe flexural limb contractures, and bone lesions. It overlaps with infantile systemic hyalinosis, which has a more severe phenotype, including visceral involvement.