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      Atypical Fibroxanthoma


 

          

Langerhans' cell histiocytosis commonly involves the head and neck, especially the skull. Presentation and extent of disease is variable. Microscopically, they consist of Langerhans cells admixed with eosinophils and macrophages. The Langerhans' cell has an indented nucleus and pink cytoplasm, is S100 and CD1a positive, and ultrastructurally contains Birbeck granules. Although the diagnosis may be suspected by the characteristic histology and strengthened by S100 positivity, demonstration of CD1a positivity or Birbeck granules is required for definite diagnosis. The differential diagnosis includes juvenile xanthogranuloma and Rosai-Dorfman disease, which may be extranodal. These conditions usually lack CD1 surface antigen and Birbeck granules.

Langerhans cell histiocytosis (Histiocytosis X)

Cranial fasciitis presents as a rapidly enlarging mass in the scalp of infants and young children.  It erodes scull bone and may extend to the dura. It is non-neoplastic and composed of stellate and spindle cells, vimentin and smooth-muscle actin positive, in myxoid stroma.There is some histological overlap with nodular fasciitis.

Nodular fasciitis

Hamartoma of the scalp with ectopic meningothelial elements consists of connective tissue with cords of plump epitheloid cells that appear to line vascular channels. 

Heterotopic Meningeal Lesions

                 

The pathology of extracranial scalp and skull masses in young children. Clin Neuropathol. 2004 Jan-Feb;23(1):34-43.

OBJECTIVE: Extracranial subcutaneous masses involving the scalp and/or skull in young children are uncommon lesions that get excised by the neurosurgeon. Although the most common reported lesion is the dermoid cyst, our experience suggests that the spectrum of pathology in these lesions can present diagnostic challenges to the pathologist. MATERIAL: We reviewed 30 consecutive extracranial masses from 29 patients between July 1998 and June 2003. METHOD: Hematoxylin and eosin-stained sections were reviewed in all cases, and immunohistochemistry was performed in select cases. RESULTS: Twenty-three were within the scalp, 5 involved the scalp and skull and 2 were within the limits of the inner and outer tables of the skull. There were 8 dermoid cysts, 2 epidermoid cysts, 6 post-traumatic lesions including 3 calcified cephalhematomas and 3 pseudocysts, 5 vascular lesions including 3 capillary hemangiomas, 1 venous angioma and 1 lymphangioma, 2 cases of cranial fasciitis and 1 case each of benign teratoma, deep granuloma annulare, benign fibrous histiocytoma, congenital melanocytic nevus, hamartoma with ectopic meningothelial elements, cutaneous hyalinised ectopic meningioma and a meningocele with a fibrohistiocytic reaction. No lesions have recurred or exhibited malignant features. CONCLUSIONS: Surgical pathologists and neuropathologists should be aware that the differential diagnosis of "lumps and bumps on babie's heads" is quite varied and can be histologically challenging.

Nodular fasciitis of the forehead in a pediatric patient. Dermatol Surg.2003 Aug;29(8):867-8.

BACKGROUND: Nodular fasciitis is a benign fibroblastic proliferation in soft tissue that is frequently misdiagnosed as a sarcoma. Most patients are middle aged, and the upper extremity is the most common localization. Nodular fasciitis is rarely diagnosed in childhood but appears in the head and neck region more commonly in children than in adults. OBJECTIVE: To describe a rare case of nodular fasciitis of the forehead in a pediatric patient. RESULTS: A 15-year-old female had been aware of a subcutaneous nodule with subtle tenderness on the forehead for 4 months. The patient had no history of trauma. The nodule was totally excised with a narrow margin and was histopathologically diagnosed as nodular fasciitis. No recurrence was observed for 22 months. CONCLUSION: When we encounter subcutaneous nodules of the face and neck region in children, it is important for dermatologists to keep nodular fasciitis in mind for differential diagnosis to avoid unnecessary wide resection.

Cranial and extracranial fasciitis of childhood: a clinicopathologic and immunohistochemical study. Hum Pathol.1999 Jan;30(1):87-92

Fasciitis of various types constitutes a distinctive category of soft tissue lesions whose microscopic features are known for their potential to evoke a pathological diagnosis of one or another type of sarcoma. Nodular fasciitis is the archetype of this group of "fibrous tumors." Cranial fasciitis is considered a nonneoplastic lesion similar to nodular fasciitis, which is seen almost exclusively in infants and children and has unique clinicopathologic features. The current study documents our experience with fibrous-myofibroblastic proliferations in extracranial sites, mainly in the head and neck region of young children, whose histological features resemble those of cranial fasciitis. These lesions were composed of loosely arranged spindle to stellate cells in a myxoid background. One patient in the study had both cranial and extracranial involvement. Some histological overlap between nodular fasciitis and cranial-extracranial fasciitis was noted, but the latter lesion tended to be more uniform in appearance than nodular fasciitis with its fascicular, spindle cell features and variability in histological patterns within the same lesion, unlike the more consistently uniform myxoid appearance of the cranial and extracranial lesions. Immunohistochemically, the spindle cells of the extracranial lesions and the one case of cranial fasciitis co-expressed vimentin and smooth muscle actin. The extracranial lesions had a predilection for children in the first year of life with all cases occurring at or before 2 years of age, unlike nodular fasciitis, which is rarely seen in the first 4 to 5 years of life. The cranial and extracranial fasciitides should be differentiated from the fibromatoses that tend to locally recur, unlike the nonrecurring behavior of these lesions in common with the other types of fasciitis.

Transcranial eosinophilic granuloma manifested by a subcutaneous scalp mass. Differential diagnosis in children.J Dermatol Surg Oncol. 1993 Jul;19(7):631-4.

BACKGROUND. The differential diagnosis of nodular scalp lesions in a child is surprisingly extensive. OBJECTIVE. To present a case of eosinophilic granuloma that was manifested by a scalp mass in a pediatric patient. METHODS. The case is presented and the relevant literature is reviewed. CONCLUSIONS. The seriousness of some of these lesions requires a thorough evaluation, including noninvasive imaging, and carefully planned surgical intervention so that effective diagnosis and treatment are ensured. Surgical exposure of unsuspected central nervous system connections may lead to infection, hemorrhage, seizures, or other significant morbidity and mortality.

Cranial fasciitis. Arch Dermatol.1989 May;125(5):674-8.

We present the clinical, roentgenographic, light-microscopic, immunohistochemical, and ultrastructural findings in two children with cranial fasciitis. A 7-year-old boy and a 3-year-old girl presented with rapidly expanding masses on the scalp. Roentgenographic studies showed erosion of the underlying cranium in one case. Both lesions showed proliferations of elongated spindle cells in a focally myxoid matrix, together with areas of hemorrhage, vascular proliferation, and chronic inflammation. Occasional cells with atypical nuclei were observed, but mitotic figures were uncommon. Immunoperoxidase studies showed negative or equivocal staining for desmin, factor VIII-associated antigen, S100 protein, and macrophage antigen. In one lesion there was focal positivity for alpha 1-antichymotrypsin, and in another lesion, some cells stained positively for smooth-muscle actin. Electron microscopy showed cells with dilated endoplasmic reticulum, bundles of microfilaments, pinocytotic vesicles, and focal external membrane material, features of myofibroblasts. Both lesions were excised and there has been no recurrence in 7 years in one case and 1 year in the other case. Cranial fasciitis is closely related to nodular fasciitis, but it has a predilection for the scalp of children. Despite its rapid growth, it has a benign clinical course and is cured by excision with or without curettage of the underlying bone. Our immunohistochemical and ultrastructural observations indicate that, like nodular fasciitis, cranial fasciitis represents a proliferation of fibroblasts and myofibroblasts.

Scalp and calvarial masses of infants and children.Neurosurgery. 1988 Jun;22(6 Pt 1):1037-42.

Review of 70 children presenting with a solitary nontraumatic lump on the head revealed that 61% of the lesions were dermoid tumor, 9% were cephalhematoma deformans, 7% were eosinophilic granuloma, and 4% were occult meningoceles and encephaloceles. Most of the dermoid cysts occurred along sutural lines, but some did not. One of the eosinophilic granulomas was located over the sagittal suture. Seventeen per cent of the "lumps" had significant intracranial extension. An additional 20% of the lumps extended intracranially, but only to the dura mater. Work-up of these lesions should include initial plain skull roentgenograms to assess multiplicity and appropriate computed tomographic scans to assess possible intracranial extension.

Cranial fasciitis of childhood.Cancer.1980 Jan 15;45(2):401-6.

Nine cases of fibroblastic lesions occurring in the cranium of young children were reviewed. The age of the patients at the time of initial treatment ranged from three weeks to six years (median 18 months), with the lesions being congenital in two cases. There was 2:1 male predominance. The size of the lesions averaged 2.5 cm in greatest dimension with the largest being 9.0 cm. All cases presented as rapidly growing masses with a preoperative duration of only two months. The lesions presented as soft-tissue masses deep in the scalp with involvement of the underlying cranium in all eight of the cases in which roentgenograms or operative reports were available for review. Characteristically, there was erosion of only the outer table of the skull, although in three cases the lesion extended through the inner table to attach to the underlying dura mater. It was not possible to detect the exact site or origin, although origin from one of the deep fascial layers of the scalp or the underlying periosteum seems most likely. Microscopically, the lesion appeared to be a proliferation of loosely arranged fibroblasts which most closely resembled nodular fasciitis. Mitotic figures as well as foci of osseous metaplasia were present. Treatment consisted of excision of the mass with local resection or curettage of the affected underlying bone in some cases. Followup revealed a benign clinical course with no recurrent or aggressive behavior.

October 2007

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