Pituitary stalk Langerhans cell histiocytosis treated with CyberKnife radiosurgery

Objective

Langerhans cell histiocytosis (LCH) is a rare idiopathic disease that is characterized by clonal proliferation of Langerhans histiocytes in various parts of the body. These atypical cells have been found to infiltrate single or multiple organs, including bone, lungs, liver, spleen, lymph nodes, and skin. Central nervous system invasion in LCH patients has rarely been reported, especially in the adult population.

Methods and results

We describe three histopathologically confirmed cases of adult LCH that involves both the pituitary stalk and hypothalamus, and report our limited experience of such cases in this location that has been treated with CyberKnife radio surgery.

Conclusion

The treatment goal of controlling lesion growth is achieved by CyberKnife radiosurgery in this case series. All patients tolerated the treatment well without obvious complications.     

巨大垂体腺瘤切除术中的垂体柄保护及意义

目的 探讨巨大型垂体腺瘤切除手术中的垂体柄保护。方法 采用经纵裂蝶窦入路显微手术切除巨大型垂体腺瘤52例。结果 肿瘤全切除39例（75％）。垂体柄保留34例（65％）。术后发生尿崩32例（62％），其中23例在术后1－2周内恢复正常，占术后尿崩总数的72％；7例在3个月内恢复；2例尿崩症状持续1年以上。结论 垂体腺瘤手术中的垂体柄保留可有效降低术后尿崩症，特别是永久性尿崩症的发生，是提高手术效果的重要措施。     

Anterior and posterior hypopituitarism with pituitary stalk abnormalities

Hypopituitarism and diabetes insipidus are often idiopathic conditions. A retrospective study of 6 cases of diabetes insipidus and 8 cases of partial or global idiopathic anterior hypopituitarism has shown that MRI is of considerable value to detect abnormalities of the pituitary stalk or hypothalamo-pituitary "relay". On the basis of MRI findings, some cases of idiopathic hypopituitarism can now be grouped together in a new entity which may be called hypopituitarism due to neonatal transection of the pituitary stalk.     

Metastasis to the pituitary stalk in a case of breast cancer

A 64-year old woman with breast cancer developed diabetes insipidus. CT scan showed thickening and contrast enhancement of the pituitary stalk highly suggestive of metastasis. The patient was successfully treated with desmopressin followed by external radiation therapy.     

Granular cell pituicytoma (choristoma) of the pituitary stalk]

A case of granular cell tumour in the pituitary stalk is described marked by the sudden loss of visual acuity in the left eye, followed by the right years later. A comparison with 17 cases previously published and with cases of asymptomatic granular cell nodules leads the authors to suggest the term granular cell pituicytoma. They discuss the place of this neoformation among other systemic granular cell tumours (granular cell myoblastoma). Fifteen patients were operated on, complete excision being carried out in only three (including the case published). Whether complementary cobalt therapy is useful remains problematical since very few cases have been published because of the slow development of the neoplasm.     

Isolated primary Langerhans' cell histiocytosis of central nervous system

Solitary eosinophilic granuloma that involves the CNS is an uncommon lesion and most frequently affects the hypothalamus. We report a new and rare case of solitary eosinophilic granuloma of the left temporal lobe in a patient without systemic disease. The diagnosis was confirmed by electron microscopy and immuohistochemical techniques. The treatment of choice is surgical resection. There is a better prognosis in comparison with cases accompanied by systemic disease.     

Distal retraction balls in the neurohypophysis after transection of the pituitary stalk

During the first few weeks after transaction of the pituitary stalk retraction balls (Cajal) were found on the fibers of the hypothalamo-neurohypophyseal nerve tract not only above but also in large numbers below the level at which the nerve tract had been cut. The latter, termed distal retraction balls, are clearly seen in routinely stained sections but are even more conspicuous after impregnation with silver. Distal retraction balls were found in the isolated neurohypophysis of man, monkey, goat and sheep. It is suggested that the development of so many retraction balls distal to the site at which the nerve tract had been severed was due largely to conditions resulting from the peculiar anatomical arrangements of the pituitary gland. Stalk section causes infarction and consequent swelling of pars distalis; this swelling, occurring in the confined space of the pituitary fossa, compresses the neurohypophysis, thereby squeezing out axoplasm from the cut ends of the isolated nerve fibers to form the distal retraction balls. Distal retraction balls were not identified with certainty in the rat, possibly because in this species the pituitary gland is not enclosed in a bony fossa, and the neurohypophysis is therefore not compressed when pars distalis swells.     

A clinico-pathological study of adult histiocytosis X involving the brain.

Adult histiocytosis X involving the CNS caused progressive spastic paraparesis. The diagnosis was made by immunoreactive anti-S100 protein antibody staining and from the presence of Birbeck granules in biopsy specimens of skin lesions. Neuropathological examination showed massive proliferation and infiltration of S-100 containing histiocyte-like cells and reactive astrocytes throughout the CNS.     

Case of histiocytosis X with a large intracranial mass

The authors experienced a case of histiocytosis X with a large intracranial mass resulting in a convulsive seizure. The patient showed left exophthalmos and a skin rash one year and two months after birth. Histiocytosis X was diagnosed from a skin biopsy, and predonine, endoxan and vincristine were administered. The rash disappeared, but the exophthalmos remained. At the age of two years and nine months, punched-out lesions appeared in the skull and 4,000 rads of radiation was applied. Thereafter, the exopthalmos persisted but there was no particular problem in the course. However, a convulsive seizure with fever suddenly appeared at nine years and ten months of age and the patient was hospitalized. At the time of admission, the general condition was good and there were no abnormalities in neurological tests. In neuroradiological examinations, a calcified and poorly vascularized mass 8 cm in maximum diameter was found to occupy the left middle cranial fossa. Chondrosarcoma was strongly suspected from these findings, but there was also symmetrical thickening of bone cortex in the peripheries of the long bones of the extremeties which appeared to be the recovery process from bone destruction caused by histiocytosis X. Therefore, the formation of an intracranial mass by histiocytosis X was diagnosed and surgery was performed. When left osteoplastic fronto-temporal craniotomy was performed, the mass was found to be raising the temporal lobe and it could be easily separated from the surrounding tissue. However, these was firm adherence to dura mater of the middle cranial fossa (especially that of the superior orbital fissure). Histologically, there were many cells with small nuclei, no polymorphism, abundant and clear cytoplasm which were darkly stained and slightly atypic. These findings matched those for histiocytosis X. Cases of histiocytosis X rarely show symptoms of the central nervous system or infiltration of the central nervous system. Only 31 such cases were seen in the literature investigated by the authors. Neurological symptoms include pyramidal symptoms such as hemiparesis and impairment of the cranial nerves, particularly paresis of the optic, trigeminal, facial and acoustic nerves. Convulsive seizures were seen in only five cases including the one reported here. It is also rare for intracranial masses to be formed in cases of histiocytosis X and only six cases, including the authors', have been found with masses of a maximum diameter of more than 5 cm.     

Isolated Langerhans cell histiocytosis of the infundibulum presenting with fulminant diabetes insipidus

Introduction Langerhans cell histiocytosis (histiocytosis X) is a rare disorder that can manifest with solitary bony lesions or disseminated disease in various tissues. A common manifestation of the disease is diabetes insipidus even in the absence of neural involvement. Disseminated disease often involves the central nervous system, but only six isolated lesions in the infundibulum have been reported.Case report An 18-year-old male had failed to develop secondary sexual characteristics. He also had diabetes insipidus for more than 1 year. Magnetic resonance imaging (MRI) of the brain showed a 1-cm homogeneously enhancing mass in the region of the infundibulum. No other lesions were found in other organ systems. The patient underwent an orbitozygomatic osteotomy and pterional craniotomy. The lesion, which was adherent to the infundibulum, was partially resected. Microscopic analysis showed multiple lymphocytes and eosinophils with Langerhans cells that stained positive for S-100. Langerhans cell histiocytosis was diagnosed, and the patient was discharged on postoperative day 6 with no neurological deficits. His diabetes insipidus persisted, and he underwent intensity-modulated radiation therapy to the sellar region.Conclusions Isolated Langerhans cell histiocytosis of the infundibulum is rare. The usual presentation is hypothalamic-pituitary axis dysfunction and diabetes insipidus. Patients with diabetes insipidus of unknown origin should undergo MRI of the sellar region to rule out infundibular abnormalities.