Pagetoid Changes Research Articles

Ossifying Fibroma; A Case report and review of literature

Abstract Introduction/Objective Ossifying fibroma (OF) is a rare, benign, true neoplasm with growth potential, with the mandible involved more often than the maxilla. There is female predilection in the third and fourth decades of life, with the most common site being the premolar and molar area of the mandible. These lesions are characterized by the replacement of endogenous bone with a highly cellular fibrous neoplasm containing varied amounts of bony trabeculae, and/or cementum-like spherules. Radiographically they present as well defined, unilocular, most are mixed lucent, opaque, some with sclerotic border and root divergence may be seen. Histologically most of the lesions are not encapsulated but are well demarcated from the adjacent bone. Thus radiographic, surgical and histological findings help distinguish OF from other benign fibro-osseous lesions such as fibrous dysplasia and cemento-osseous dysplasia. Distinguishing an accurate diagnosis between the above fibro-osseous neoplasms becomes significant as prognosis and treatment differ. Methods/Case Report Herein we report a case of a 59-year-old female who presented with a symptomatic mandibular lesion that radiographically illustrated a midline well-circumscribed expansile mass of the mandibular symphysis with a sclerotic margin and ground-glass internal matrix measuring 3.1 x 4.9 x 3.9 cm, favored to represent pagetoid changes more likely than neoplastic process. Microscopic examination revealed numerous variably sized islands of ossification within a hypercellular fibrous stroma. Based on the clinical, radiographic and histologic findings, a diagnosis of benign fibro-osseous lesion, favor ossifying fibroma was given. Results (if a Case Study enter NA) NA Conclusion Microscopic examination, as in our case, can resemble a variety of benign fibro-osseous neoplasms. Radiographic and clinical correlation, along with microscopic evaluation, help solidify the accurate diagnosis.

Extramammary Paget's Disease of the Vulva: A Case Report and Review of the Literature

Abstract Background: Extramammary Paget's disease (EMPD) of the vulva is a rare clinical entity that often affects Caucasian postmenopausal women. The disease presents with vulval itching and soreness. Wide local excision, topical application of chemotherapeutic agents, and radiotherapy have been used to treat this rare condition. Case: A 65-year-old, para 3, postmenopausal woman presented with vulval itching and burning, which topical dermatologic creams failed to resolve. Local and pelvic examinations did not show any abnormality but a biopsy showed that she had Paget cells along the basal part of the squamous epithelium of the skin. The affected area was excised and the removed specimen was examined histologically, showing Pagetoid changes. This patient was treated with imiquimod cream for 6 months. Results: At a 6-month follow-up, this patient was symptom-free and had no clinical evidence of a recurrence. Conclusions: EMPD has a propensity for recurrence and, rarely, may signal an underlying adenocarc...

Pagetoid Adenocarcinoma In Situ of the Cervix With Pagetoid Spread Into the Vagina

Pagetoid spread of adenocarcinoma in situ of the cervix into vaginal epithelium is a rare phenomenon, mimicking Paget's disease of the vulva. We report a case of in situ squamous cell and in situ adenocarcinoma of the cervix in a 44-year-old woman who underwent total abdominal hysterectomy, bilateral salpingo-oophorectomy, and upper vaginectomy. Pathologic evaluation showed pagetoid endocervical adenocarcinoma in situ spreading into the vagina with positive margins. Malignant cells were positive for mucin, P16, CK-7, and carcinoembryonic antigen. The asymptomatic occult finding of pagetoid changes in the vagina should raise the suspicion of vaginal extension of pagetoid genital or urothelial carcinoma in situ; however, it can be mistaken as vaginal dysplasia or primary Paget's disease. Immunohistochemical profiling may help in the differential diagnosis.

Intracranial extension of meibomian gland carcinoma with pagetoid changes

A 41-year-old man presented with exophthalmos and loss of visual acuity. Neuroradiological studies showed a large mass extending from the intraorbital region to the frontal lobe. In addition, it also involved the middle cranial and infratemporal fossae with accompanying skull destruction. The tumor was almost totally removed. The histological diagnosis was sebaceous carcinoma with pagetoid changes. Despite surgery and local irradiation, intracranial metastases were recognized one year later, and the patient underwent total tumor removal and whole-brain irradiation. Although multiple lung metastases were detected one year after the second operation, three years post-surgery he remains free of intracranial tumor recurrence. Sebaceous carcinoma of meibomian gland origin with pagetoid changes is a distinct, highly aggressive clinical entity. Early diagnosis and appropriate treatment are essential to improve the prognosis of patients with meibomian gland carcinoma with intracranial extension.

Radiology Quiz Case 4

A 27-YEAR-OLD BOY PRESENTED WITH HEARing loss. He had a 20-year history of progressive bilateral visual loss. Bilateral decompression of the optic nerves had been performed 10 years earlier, with no improvement in visual acuity. The patient, who developed precocious puberty when he was 10 years old, also had abnormal skin pigmentation and epilepsy. Physical examination revealed a progressive “football-like” deformity of the cranial cavity and pathologic fractures of the legs that had occurred after minimal trauma. Because the patient’s external auditory canals were very narrow, the tympanic membrane could not be explored in either ear. Tonal audiometry showed normal hearing levels in the right ear and a mild conductive hearing loss in the left ear. A computed tomographic scan showed diffuse involvement of the skull base, with a heterogeneous pattern of sclerotic, cystic, and pagetoid changes. Both temporal bones were irregular and asymmetrically affected, with only narrow portions of perilabyrinthine bone involved (Figures 1, 2, and 3). What is your diagnosis?

The pagetoid variant of bladder urothelial carcinoma in situ A clinicopathological study of 11 cases.

Pagetoid urothelial carcinoma in situ (CIS) is a rare variant of bladder cancer that is characterized by an intraepithelial proliferation of large cells arranged singly or in clusters and randomly distributed. These neoplasms deserve recognition and attention, chiefly because they may be overlooked or misdiagnosed as urothelial dysplasia, then causing unsuspected tumor recurrence after surgery. We report on the clinicopathological features and immunohistochemical findings of 11 (14.86%) cases of pagetoid CIS in a retrospective study of 74 cases of conventional carcinoma in situ. Most patients were male ( n=10). Their ages ranged from 31 years to 78 years. The lesion can be present with primary ( n=2) or secondary ( n=9) CIS. Pagetoid CIS is usually a focal lesion occurring in a clinical and histological setting of conventional CIS, and these patients essentially have the same progression and survival rates as patients without pagetoid changes and are treated in the same way. In cases with extensive urothelial denudation, pagetoid CIS may be focally present in otherwise normal-looking urothelium, thus alerting the pathologist to search for additional CIS elsewhere in the bladder. Given that primary extramammary Paget disease of the external genitalia and of the anal canal may extend to the bladder and, conversely, some bladder cases of pagetoid CIS may extend to the urethra, ureter, and beyond to the external genitalia, the differential diagnoses between these two entities represent an important therapeutic consideration. Our data suggest that a panel of immunostains including CK7+/CK20+/TM+ may assist in differentiating urothelial pagetoid CIS from extramammary Paget disease which is known to be CK7+/CK20-.

Atypical presentation of Paget's disease on bone scintigraphy

A 76-year-old man with a history of ankylosing spondylitis (AS) was evaluated for possible Paget's disease (PD) based on an elevated alkaline phosphatase level of 197 U/I (normal, 36 to 126 U/I) and a urinary hydroxyproline level of 68 mg per 24 hours (normal, 5 to 65 mg). Roentgenograms of the posteroanterior and lateral chest, lateral lumbar spine, and sacroiliac joints revealed marked kyphosis within the thoracic spine and a flowing anterior syndesmophyte and ankylosis of the sacroiliac joints consistent with AS. The films also showed expansion, prominent trabecula, end cortical thickening of affected thoracic ribs, and L2-L3 vertebral bodies, consistent with PD. Additional changes of PD were seen in the T7-T10 vertebral bodies and the anterior syndesmophyte bridging these levels. Anterior and posterior whole-body scintiscans were obtained after intravenous administration of 25 mCi Tc-99m MDP. The scintigrams showed typical increased uptake in the T7-T11 and L2-L3 vertebrae, consistent with PD. In addition, they demonstrated increased uptake in the posterior seventh through tenth ribs bilaterally, corresponding in location to the pagetoid changes seen on the roentgenograms. This bone scan appearance is unusual in that unlike the typical PD presentation (i.e., extension distally from the articular surface of the bone without crossing of the joint), PD in this case extended across joints fused by AS.

The pagetoid variant of urothelial carcinoma in situ

The frequency, distribution, and clinical significance of pagetoid changes in urothelial carcinoma in situ (CIS) were studied in over 600 tissue slides from 102 patients followed for a mean period of over 4 years. Results indicate that pagetoid changes are unusual, randomly distributed, and never widespread. These changes occur in a histologic setting of CIS and apparently never appear as the initial lesion, even in cases in which CIS is the primary or only neoplasm. Patients manifesting pagetoid CIS had essentially the same progression and survival rates as patients without pagetoid changes. Our data suggest that this histologic pattern represents a peculiar reaction to injury confined to small areas of urothelium in susceptible hosts.

DNA flow cytometry of sebaceous cell carcinomas of the ocular adnexa: introduction to the technique in the evaluation of periocular tumors.

DNA content abnormalities are well recognized in tumor cell biology and kinetics. We introduce the technique of DNA flow cytometry through the study of the sebaceous cell carcinomas of the ocular adnexa. By correlating the data to the standard histopathologic parameters of pagetoid changes, degree of anaplasia, and stromal inflammation, significant associations are revealed. All aneuploid tumors demonstrate pagetoid spread, and more severe anaplasia and stromal inflammation. All diploid tumors are nonpagetoid and have lesser degrees of anaplasia and stromal inflammation. A complete review of the technique with a discussion of the implications and applicability to the study of ocular adnexal tumors is presented.

AN UNUSUAL CASE OF EXTRAMAMMARY PAGET'S DISEASE

A patient, having been treated by total cystectomy and Bricker's conduit operation for prostatic duct carcinoma with intraepithelial extension into the prostatic urethra and bladder, developed an annular, psoriasiform eruption around the external orifice of the urethra two and one-half years later. A biopsy of this lesion showed pagetoid changes of the epidermis. Microscopic examination of the surgical specimen, consisting of the glans penis and corpus spongiosum, demonstrated intraepithelial-tumor permeation into the glans penis, penile urethra, and periurethral glands (Littre). It seems reasonable to conclude that this is an unusual case of Paget's disease of the glans penis which probably originated from a prostatic duct carcinoma.

CARCINOMA OF SEBACEOUS GLANDS OF THE EYELID.

This paper reports a patient with an unusual form of diffuse carcinoma of the meibomian glands and the glands of Zeis. Although carcinoma of the meibomian glands classically is characterized by a discrete lesion often mistaken for a chalazion, 1 this patient showed only a generalized thickening and inflammation in the lateral portions of the upper and lower lids. The diagnosis was confirmed by biopsy which further revealed pagetoid changes in the overlying epithelium. Report of Case This 72-year-old white male was seen in consultation on Sept 4, 1963. He had noticed pain and irritation on the lateral aspects of the eyelids of his left eye for one year. Treatment with antibiotic and steroid ointments had resulted in no improvement. Examination revealed a diffuse, apparently inflammatory, eczematoid thickening of the temporal 1.5 cm of the left upper and lower lids adjacent to the outer canthus of the left eye (Fig