Reith J D, Goldblum J R
Department of Anatomic Pathology, The Cleveland Clinic Foundation, OH 44195, USA.
Arch Pathol Lab Med. 1996 Apr;120(4):399-401.
Plexiform schwannomas are relatively rare, benign peripheral nerve sheath tumors, which usually arise in either the dermis or subcutaneous tissue, although rare cases originate in skeletal muscle or other deep somatic soft tissue sites. These tumors may occur singly or as multiple lesions and may be localized to one anatomic site or diffusely distributed. Rare cases have been associated with "schwannomatosis" as well as type 1 neurofibromatosis (von Recklinghausen's disease). We report an unusual case of multiple cutaneous plexiform schwannomas associated with bilateral acoustic neuromas as well as other intracranial and intraspinal neoplasms. In addition, we examine the relationship between the various forms of cutaneous schwannoma, particularly the plexiform variant, and both types 1 and 2 neurofibromatosis; we also examine several purported cases of schwannomatosis.
丛状神经鞘瘤是相对罕见的良性周围神经鞘瘤，通常发生于真皮或皮下组织，不过也有罕见病例起源于骨骼肌或其他深部躯体软组织部位。这些肿瘤可单发或多发，可局限于一个解剖部位，也可呈弥漫性分布。罕见病例与“神经鞘瘤病”以及1型神经纤维瘤病（冯雷克林霍增氏病）有关。我们报告了一例不寻常的病例，该病例为多发皮肤丛状神经鞘瘤，伴有双侧听神经瘤以及其他颅内和椎管内肿瘤。此外，我们研究了各种皮肤神经鞘瘤，尤其是丛状变型，与1型和2型神经纤维瘤病之间的关系；我们还研究了几例所谓的神经鞘瘤病病例。
