Unifocal Langerhans' cell histiocytosis and frontal sinus agenesis: report of a rare case.Abstract
Unifocal Langerhans' cell histiocytosis Langerhans' cell histiocytosis Histiocytosis X, Langerhans cell granulomatosis, multifocal eosinophilic granuloma Hematology An autonomous proliferation of a lymphoreticular cell, the Langerhans cell, which stains positively with antibodies to ATPase, S-100 and of the frontal bone is rare. We report a most unusual case of Langerhans' cell histiocytosis of the frontal bone in a patient who had been affected by frontal sinus agenesis agenesis
Failure of all or part of an organ to develop during embryonic growth. Many forms of agenesis are lethal, such as absence of the entire brain (anencephaly), but agenesis of one organ of a pair may cause little problem. . We believe this is the first reported case of its kind. We also review previously reported cases of solitary eosinophilic granuloma of the frontal bone and we discuss its clinical, radiologic, and histopathologic features.
Langerhans' cell histiocytosis usually occurs during childhood. The lesions can involve bones in isolation or as a part of disseminated disease. Unifocal Langerhans' cell histiocytosis of the skull is rare; when it has occurred, it has been reported in the frontal, temporal, and sphenoid bones and the mandible. (1-6) In this article, we describe what to our knowledge is the first reported case of unifocal Langerhans' cell histiocytosis in a frontal bone that had been affected by frontal sinus agenesis.
A 17-year-old girl came to our department with a 2-week history of headache and increasing central forehead swelling (figure 1). The area of swelling, which measured approximately 5 x 4cm, was soft and tender to palpation palpation /pal·pa·tion/ (pal-pa´shun) the act of feeling with the hand; the application of the fingers with light pressure to the surface of the body for the purpose of determining the condition of the parts beneath in physical diagnosis. . The patient was otherwise well, her constitutional signs were normal, and the results of routine hematologic hematological, hematologic
pertaining to or emanating from blood cells.
total and differential white cell counts, hematocrit estimation, erythrocyte count. investigations were unremarkable.
Computed tomography (CT) of the paranasal sinuses revealed the presence of frontal sinus agenesis and a well-defined, low-density lesion in the diploe dip·lo·e
The central layer of spongy, porous, bony tissue between the hard outer and inner bone layers of the cranium.
diplo·ic adj. of the frontal bone (figure 2). A small, nonenhancing, high-density area was noted within the low-density lesion. The outer table of the frontal bone was completely eroded in the midline, and associated attenuation of the inner cortex was visible. Fine-needle aspiration cytology was nondiagnostic, as it detected only scant inflammatory cells and no evidence of malignancy. The patient was admitted to the hospital.
After a course of intravenous amoxicillin/clavulanate, the area of swelling decreased rapidly, and the patient was discharged 2 days after admission and prescribed oral antibiotics. She was readmitted 1 week later for elective bicoronal-flap surgical exploration. Intraoperatively, a soft-tissue mass was found within a central defect in the frontal bone; the mass extended deep to the aponeurosis aponeurosis /ap·o·neu·ro·sis/ (-ndbobr-ro´sis) pl. aponeuro´ses [Gr.] a sheetlike tendinous expansion, mainly serving to connect a muscle with the parts it moves. (figure 3). The mass was completely excised and the bony defect was smoothed with a diamond drill. The defect was obliterated with a locally harvested free bone graft. The patient's postoperative recovery was uneventful.
Histopathologic examination of the lesion identified bony trabeculae and loosely textured fibrous tissue that contained many plump histiocytic histiocytic
pertaining to histiocytes.
see malignant histiocytosis.
prolymphocyte. cells, each with an oval, vesicular, occasionally grooved nucleus and abundant, pale-staining cytoplasm (figure 4). Eosinophils Eosinophils
A leukocyte with coarse, round granules present.
Mentioned in: Histiocytosis X
eosinophils were numerous in the background, together with dispersed plasma cells and lymphocytes, many of which formed clusters. There were areas of necrosis associated with neutrophils. Immunohistochemical staining for S-100 protein and CD1a confirmed that the histiocytic cells were Langerhans' cells. A radionucleotide bone scan performed to look for other lesions was clear. At the 1-year review, the patient remained asymptomatic and free of disease.
The frontal sinus is absent at birth. It begins to develop at 6 to 12 months of age. Each frontal sinus is made up of two independent spaces that develop from different sources. One is formed by the expansion of the ethmoid sinus into the frontal bone, and the other develops as a result of an independent invagination invagination /in·vag·i·na·tion/ (in-vaj?i-na´shun)
1. the infolding of one part within another part of a structure, as of the blastula during gastrulation.
2. intussusception. of the middle meatus of the nasal passage. These developments occur at approximately 5 to 6 years of age, and the sinuses continue to expand throughout adolescence. (7)
Very little has been written about the factors that affect the growth of these sinuses or the factors that cause agenesis. We do know that frontal sinus agenesis has been reported in 1% of the British population as a whole, (8) in 5 to 9% of patients with sinusitis, (9, 10) and in as many as 63% of patients who have cystic fibrosis. (11)
Langerhans' cell histiocytosis--also known as histiocytosis histiocytosis /his·tio·cy·to·sis/ (-si-to´sis) a condition marked by an abnormal appearance of histiocytes in the blood.
acute disseminated Langerhans cell histiocytosis Letterer-Siwe disease. X--is primarily a pediatric disease. Its incidence has been reported to be 3 to 4 cases per 1 million children. (1) It is a poorly understood proliferative disease of Langerhans' cells, which are normally present in the epidermis of the skin and related to the mononuclear phagocyte system mononuclear phagocyte system
Abbr. MPS A widely distributed collection of both free and fixed macrophages derived from bone marrow precursor cells by way of monocytes; their substantial phagocytic activity is mediated by . Langerhans' cell histiocytosis can manifest as three distinct clinicopathologic entities: acute disseminated Langerhans' histiocytosis (previously known as Letterer-Siwe disease), unifocal Langerhans' cell histiocytosis (previously known as eosinophilic granuloma). and multifocal multifocal /mul·ti·fo·cal/ (mul?te-fo´k'l) arising from or pertaining to many foci.
Relating to or arising from many foci. Langerhans' cell histiocytosis (previously known as Hand-Schuller-Christian disease).
The acute disseminated variety affects infants and children and carries a high mortality rate. The unifocal and multifocal variants run a more benign course, and both are characterized by expanding and erosive accumulations of Langerhans' cell histiocytes, usually in the medullary cavities of the bones. In such cases, the histiocytes are mixed with eosinophils, lymphocytes, plasma cells, and neutrophils. The most common sites of all types of Langerhans' cell histiocytosis are the calvaria calvaria /cal·va·ria/ (kal-var´e-ah) [L.] the domelike superior portion of the cranium, comprising the superior portions of the frontal, parietal, and occipital bones.
n. pl. , ribs, and femur, although virtually any bone can be affected. Similar lesions can be found on the skin, lungs, or stomach, either as unifocal disease or as components of the multifocal variety.
Unifocal Langerhans' cell histiocytosis is an indolent disorder that affects children and young adults, and it is more common in males. The manifestation of solitary bone lesions can range from asymptomatic to painful swelling or pathologic fracture. There are no systemic features and no involvement of the blood or viscera viscera /vis·ce·ra/ (vis´er-ah) plural of viscus.
1. The soft internal organs of the body, especially those contained within the abdominal and thoracic cavities. . The results of hematologic tests are usually unremarkable, although eosinophilia eosinophilia /eo·sin·o·phil·ia/ (e?o-sin?o-fil´e-ah) abnormally increased eosinophils in the blood.
An increase in the number of eosinophils in the blood. might be present. (12)
Plain x-rays might detect lytic lytic /lyt·ic/ (lit´ik)
1. pertaining to lysis or to a lysin.
2. producing lysis.
1. Of, relating to, or causing lysis.
2. lesions in affected bone, along with surrounding sclerosis if the defect has begun to heal. (13) CT will detect lytic areas within the diploe that contain soft tissue; the soft tissue is usually homogeneous, but it can include a bony sequestrum sequestrum /se·ques·trum/ (se-kwes´trum) pl. seques´tra [L.]
1. any sequestered tissue.
2. a piece of dead bone separated from the sound bone in necrosis. . The lesions might or might not enhance with contrast, and the margins of the bone might or might not demonstrate sclerosis. Magnetic resonance images will be of variable signal intensity (isoto hypointense) and will demonstrate gray or white matter on T1-weighted imaging and a strong signal intensity (hyperintense) on T2-weighted imaging. The lesions as a whole enhance markedly after administration of gado linium, although some areas within the lesion do not enhance well; the surrounding dura, galea galea /ga·lea/ (ga´le-ah) [L.] a helmet-shaped structure.
galea aponeuro´tica the aponeurosis connecting the two bellies of the occipitofrontalis muscle. , and muscle might also enhance. (14)
The diagnosis is usually based on histologic examination of incisional or excisional biopsy specimens; radiology is not diagnostic. (14) Once the diagnosis has been established, radionucleotide bone scintigraphy scintigraphy /scin·tig·ra·phy/ (sin-tig´rah-fe) the production of two-dimensional images of the distribution of radioactivity in tissues after the internal administration of a radiopharmaceutical imaging agent, the images being obtained should be carried out to look for other bony lesions. Histology will reveal the presence of histiocytes, eosinophils, plasma cells, lymphocytes, and neutrophils. The histologic picture is sometimes difficult to differentiate from that of osteomyelitis because of the presence of macrophages in the background. (15) The sighting of Birbeck granules (also known as HX bodies) in the cytoplasm of Langerhans' cell histiocytes on electron microscopy is pathognomonic pathognomonic /pa·thog·no·mon·ic/ (path?ug-no-mon´ik) specifically distinctive or characteristic of a disease or pathologic condition; denoting a sign or symptom on which a diagnosis can be made. . Birbeck granules are microtubular structures shaped like tennis rackets. Immunohistochemical staining will demonstrate the presence of S-100 protein in these macrophages. (16)
The treatment of Langerhans' cell histiocytosis depends on its clinical subgroup. Unifocal lesions sometimes undergo fibrosis and heal spontaneously; sometimes an incisional biopsy is sufficient to trigger healing. Local excision and repair of bony defects with bone grafts, excision of soft-tissue lesions, radiotherapy, chemotherapy, and immunotherapy (for disseminated disease) have all been described. Although some studies have looked at the relative efficacy of one type of treatment over others, particularly in patients with the disseminated variety of Langerhans' cell histiocytosis, there is no evidence in the literature to suggest that any one modality is superior. (17,18)
(1.) Irving RM, Broadbent V, Jones NS. Langerhans' cell histiocytosis in childhood: Management of head and neck manifestations. Laryngoscope 1994; 104:64-70.
(2.) Moshegov C, Martin P, Myers P, Filipic M. Langerhans' cell histiocytosis of the frontal bone. Aust N Z J Ophthalmol 1994;22:133-8.
(3.) Stromberg JS, Wang AM, Huang TE, et al. Langerhans cell histiocytosis involving the sphenoid sinus and superior orbital fissure superior orbital fissure
A cleft between the greater and the lesser wing of the sphenoid, through which pass the oculomotor and trochlear nerves, the ophthalmic division of the trigeminal nerve, the abducens nerve, and the ophthalmic veins. . AJNR Am J Neuroradiol 1995;16(4 Suppl):964-7.
(4.) Jakobiec FA, Trokel SL, Aron-Rosa D, et al. Localized eosinophilic granuloma (Langerhans' cell histiocytosis) of the orbital frontal bone. Arch Ophthalmol 1980;98:1814-20.
(5.) Goldsmith AJ, Myssiorek D, Valderrama E, Patel M. Unifocal Langerhans' cell histiocytosis (cosinophilic granuloma granuloma /gran·u·lo·ma/ (gran?u-lo´mah) pl. granulomas, granulo´mata an imprecise term for (1) any small nodular delimited aggregation of mononuclear inflammatory cells, or (2) such a collection of modified macrophages ) of the petrous petrous /pet·rous/ (pet´rus) resembling a rock; hard; stony.
1. Of stony hardness.
2. apex. Arch Otolaryngol Head Neck Surg 1993;119:113-6.
(6.) Schepman KP, Radden BG, Van der Waal I. Langerhans' cell histiocytosis of the jaw bones. Report of 11 cases. Aust Dent J 1998;43:238-41.
(7.) Larsen WJ. Development of head and neck. In: Larsen WJ. Human Embryology. 2nd ed. New York: Churchill Livingstone, 1997:366.
(8.) Lund VJ. Anatomy of the nose and paranasal sinuses. In: Kerr AG, ed. Scott-Brown's Otolaryngology. 6th ed. Vol. 1. Oxford: Butterowrth-Heinemann, 1997;chap. 5:24.
(9.) Duvoisin B, Schnyder P. Do abnormalities of the frontonasal duct cause frontal sinusitis? A CT study in 198 patients. AJR Am J Roentgenol 1992;159:1295-8.
(10.) Amedee R. Sinus anatomy and function. In: Bailey BJ, ed. Head and NeckSurgery: Otolaryngology. Philadelphia: J.B. Lippincott, 1993:342-9.
(11.) Nishioka GJ, Cook PR, McKinsey JP, Rodriguez FJ. Paranasal sinus computed tomography scan Computed tomography scan (CT scan)
A specialized type of x-ray imaging that uses highly focused and relatively low energy radiation to produce detailed two-dimensional images of soft tissue structures, particularly the brain. findings in patients with cystic fibrosis. Otolaryngol Head Neck Surg 1996;114:394-9.
(12.) Kumar V. Cotran RS, Robbins SL. Hematopoietic hematopoietic /he·ma·to·poi·et·ic/ (-poi-et´ik)
1. pertaining to hematopoiesis.
2. an agent that promotes hematopoiesis.
1. pertaining to or affecting the formation of blood cells. and lymphoid systems. In: Kumar V, Cotran RS, Robbins SL, eds. Basic Pathology. 6th ed. Philadelphia: W.B. Saunders, 1997:340-92.
(13.) Broadbent V, Egeler RM, Nesbit ME, Jr. Langerhans' cell histiocytosis--clinical and epidemiological aspects. Br J Cancer Suppl 1994;23:S11-6.
(14.) Marioni G, De Filippis C, Starmare R, et al. Langerhans cell histiocytosis: Temporal bone involvement. J Laryngol Otol 2001;115:839-41.
(15.) Schiller AL. Bones and joints. In: Robin E, Farber JL, eds. Pathology. Philadelphia: J.B. Lippincott, 1988: 1304-93.
(16.) Eriksen B, Janinis J, Variakojis D, et al. Primary histiocytosis X of the parieto-occipital lobe. Hum Pathol 1988;19:611-4.
(17.) Ladisch S, Gadner H. Treatment of Langerhans cell histiocytosis--evolution and current approaches. Br J Cancer Suppl 1994;23:S41-6.
(18.) McLelland J, Pritchard J, Chu AC. Current controversies. Histiocytosis-X. Hematol Oncol Clin North Am 1987;1:147-62.
From the Department of Otorhinolaryngology (Dr. Basha and Dr. Kinsella) and the Dcpartmcnt of Pathology (Dr. Napier), the Royal Victoria Hospital, Belfast The Royal Victoria Hospital (Irish: Ospidéal Ríoga Victoria; commonly known as "The Royal" or the "RVH") is a hospital in Belfast, Northern Ireland.
The hospital, which provides over a fifth of the acute beds in Northern Ireland and treats half a million patients a , Northern Ireland, U.K.
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