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Giant condyloma acuminatum (also known as a Buschke–Löwenstein tumor and "Giant condyloma of Buschke–Löwenstein tumor") is a rare cutaneous condition characterized by an aggressive, wart-like growth that is a verrucous carcinoma. It is attributed to human papillomavirus.
Neurocutaneous melanosis is associated with the presence of either giant congenital melanocytic nevi or non-giant nevi of the skin. It is estimated that neurocutaneous melanosis is present in 2% to 45% of patients with giant congenital melanocytic nevi. Patients with non-giant congenital melanocytic nevi seem to have a much lower, but undefined risk. Of these patients, only a small number are symptomatic, usually displaying symptoms before the age of 2.
These symptoms are the result of melanocytic lesions being present in the leptomeninges of the central nervous system.
Symptoms can include:
- Papilledema
- Cranial palsies
- Headache
- Vomiting
- Seizures
Others symptoms may also exist that are related to an increase in intracranial pressure. These symptoms seem to be present regardless of the malignancy of the melanin deposits within the central nervous system.
Approximately 10% of patient with neurocutaneous melanosis also present the Dandy–Walker syndrome and associated Dandy-Walker malformation. This malformation involves an enlargement of the posterior fossae and fourth ventricle along with agenesis of the cerebellar vermis. The abnormalities of the leptomeninges during fetal development due to neurocutaneous melanosis may be the cause of this increased incidence of the Dandy-Walker malformation. The development of hydrocephalus is the most common symptom associated with a combination of neurocutaneous melanosis and a Dandy-Walker malformation, occurring in about two out of three patients.
The congenital melanocytic nevus appears as a circumscribed, light brown to black patch or plaque, potentially very heterogeneous in consistency, covering any size surface area and any part of the body.
As compared with a melanocytic nevus, congenital melanocytic nevi are usually larger in diameter and may have excess terminal hair, a condition called hypertrichosis. If over 40 cm projected adult diameter with hypertrichosis, it is sometimes called giant hairy nevus; more usually these largest forms are known as large or giant congenital melanocytic nevus. The estimated prevalence for the largest forms is 0.002% of births.
Melanocytic Nevi often grow proportionally to the body size as the child matures. As they mature, they often develop thickness, and become elevated, although these features can also be present from birth. Prominent terminal hairs often form, especially after puberty. With maturity, the nevus can have variation in color, and the surface might be textured with proliferative growths.
Neurocutaneous melanosis is associated with the presence of either giant congenital melanocytic nevi or non-giant nevi of the skin. It is estimated that neurocutaneous melanosis is present in 2% to 45% of patients with giant congenital melanocytic nevi. Neurocutaneous melanosis is characterized by the presence of congenital melanocytic nevi on the skin and melanocytic tumors in the leptomeninges of the central nervous system.
Congenital melanocytic nevi may be divided into the following types:
- "Small-sized congenital melanocytic nevus" is defined as having a diameter less than 2 cm.
- "Medium-sized congenital melanocytic nevus" is defined as having a diameter more than 2 cm but less than 20 cm.
- "Giant congenital melanocytic nevus" (also known as "Bathing trunk nevus," "Garment nevus," "Giant hairy nevus", and "Nevus pigmentosus et pilosus") is defined by one or more large, darkly pigmented and sometimes hairy patches.
Giant-cell fibroma is a type of fibroma not associated with trauma or irritation. It can occur at any age and on a mucous membrane surface. The most common oral locations are on the gingiva of the mandible, tongue, and palate. It is a localized reactive proliferation of fibrous connective tissue.
Giant-cell fibroma (GCF) is a benign non-neoplastic lesion first described by Weathers and Callihan (1974). It occurs in the first three decades of life and predominates in females (Houston, 1982; Bakos, 1992). Clinically, the GCF presents as an asymptomatic, papillary and pedunculated lesion. The most predominant location is the mandibular gingiva (Houston, 1982; Bakos, 1992). Histologically, the GCF is distinctive, consisting of fibrous connective tissue without inflammation and covered with stratified squamous hyperplastic epithelium. The most characteristic histological feature is the presence of large spindle-shaped and stellate-shaped mononuclear cells and multinucleated cells. These cells occur in a variety of lesions, such as the fibrous papule of the nose, ungual fibroma, acral fibrokeratoma, acral angiofibroma and desmoplastic fibroblastoma (Swan, 1988; Pitt et al., 1993; Karabela-Bouropoulou et al., 1999; Jang et al., 1999).
Despite many studies, the nature of the stellated multinucleate and mononuclear cell is not clear (Weathers and Campbell, 1974; Regezi et al., 1987; Odell et al., 1994; Magnusson and Rasmusson, 1995).
Differential diagnosis includes seborrheic keratosis, verruca simplex, condyloma acuminatum, granular cell myoblastoma, vulvar intraepithelial neoplasia, bowenoid papulosis, erythroplasia of Queyrat, and verrucous carcinoma
The afflicted may have relatively small amounts of pain that will quickly increase in severity over a time period of 6–12 weeks. The skin temperature around the bone may increase, a bony swelling may be evident, and movement may be restricted in adjacent joints.
Spinal lesions may cause quadriplegia and patients with skull lesions may have headaches.
CGCG lesions are found more commonly in the anterior of the maxilla and the mandible in younger people (before age 20). They are characterized by large lesions that expand the cortical plate and can resorb roots and move teeth. They are composed of multi-nucleated giant cells. CGCG has a slight predilection for females.
Radiographically :
It appears as multilocular radiolucencies of bone.The margin of the lesion has scalloped appearance and is well demarcated. Resorption and divergence of roots is also seen.
There are two types of CGCG's, non-aggressive and aggressive. The former has a slow rate of growth and thus less likely to resorb roots and perforate the cortical plate. The aggressive form has rapid growth and thus is much more likely to resorb roots and perforate the cortical plate. It also has a high rate for recurrence and can be painful and cause paresthesia.
Differential diagnosis to include: odontogenic keratocyst (OKC), ameloblastoma, odontogenic myxoma, hemangioma, central odontogenic fibroma, hyperparathyroid tumor, and cherubism.
Giant-cell tumor of the tendon sheath, also known as giant-cell synovioma and localized nodular tenosynovitis, is a firm lesion, measuring 1 to 3 cm in diameter, and is most commonly attached to the tendons of the fingers, hands, and wrists, with a predilection for the flexor surfaces. Giant-cell tumor of tendon sheaths most often affect the wrist and fingers of males and females from the ages of 20-50 . These tumors are typically painless and can cause cortical erosion. Surgery to remove the tumor is a common treatment, though the tumors tend to recur.
The most common location by far is the gingival margin and other areas of the masticatory oral mucosa, these occur more frequently in the fifth decade of life, and have good prognosis, the treatment of choice for oral VXs is surgical excision, and recurrence is rare.
The condition can affect other organs of body, such as the penis, vulva, and can occur in anal region, nose, the ear, lower extremity, scrotum.
Central giant-cell granuloma (CGCG) is a benign condition of the jaws. It is twice as likely to affect women and is more likely to occur in 20- to 40-year-old people. Central giant-cell granulomas are more common in the mandible and often cross the midline.
Warty dyskeratoma, also known as an Isolated dyskeratosis follicularis, is a benign epidermal proliferation with distinctive histologic findings that may mimic invasive squamous cell carcinoma and commonly manifests as an umbilicated (Having a central mark or depression resembling a navel) lesion with a keratotic plug, WD have some histopathologic similarities to viral warts but it's not caused by HPV and the majority of these lesions display overall histopathologic features consistent with a follicular adnexal neoplasm. usually limited to the head, neck, scalp or face and vulva. Lesions are generally and sporadic and may be associated with a follicular unit. Oral involvement, particularly the hard palate, and genital involvement have been reported. it can also be thought of as one of the manifestations of focal acantholytic dyskeratosis, an epidermal reaction pattern that can be seen in several disorders, including Darier's disease and Grover's disease. But the main Difference between Darier disease and Warty dyskeratoma, is that Darier disease inherited dermatosis (autosomal dominant) consisting of multiple keratotic papules on the face, trunk, and extremities, while WD occurs as an isolated, noninherited, single keratotic nodule mainly confined to the head and neck as mentioned earlier.
PCNA and Ki67 immunoreactivity happens in case of fibroma and peripheral granuloma.
Neurocutaneous melanosis is a congenital disorder characterized by the presence of congenital melanocytic nevi on the skin and melanocytic tumors in the leptomeninges of the central nervous system. These lesions may occur in the amygdala, cerebellum, cerebrum, pons, and spinal cord of patients. Although typically asymptomatic, malignancy occurs in the form of leptomeningeal melanoma in over half of patients. Regardless of the presence of malignancy, patients with symptomatic neurocutaneous melanosis generally have a poor prognosis with few treatment options. The pathogenesis of neurocutaneous melanosis is believed to be related to the abnormal postzygotic development of melanoblasts and mutations of the NRAS gene.
Warty dyskeratoma must be differentiated from vulvar dysplasia, Bowenoid papulosis, squamous carcinoma, condyloma, and other viral-induced squamous lesions.
Aneurysmal bone cyst, abbreviated ABC, is an osteolytic bone neoplasm characterized by several sponge-like blood or serum filled, generally non-endothelialized spaces of various diameters.
The term is a misnomer, as the lesion is neither an aneurysm nor a cyst.
Pilomatricoma, also known as a calcifying epithelioma of Malherbe, Malherbe calcifying epithelioma, and Pilomatrixoma, is a benign skin tumor derived from the hair matrix. These neoplasms are relatively uncommon and typically occur on the scalp, face, and upper extremities. Clinically, pilomatricomas present as a subcutaneous nodule or cyst with unremarkable overlying epidermis that can range in size from 0.5-3.0 cm, but the largest reported case was 24 cm.
The most common symptom is mild to severe pain that is gradually progressive in the affected region and may be initially attributed to a minor injury or sports-related injury. Pain may be present for several weeks, months, or years. Other symptoms in order of most common to least commonly observed include swelling, a limp (when affected bone is in the lower extremity), joint stiffness, and a soft tissue mass.
Physical findings include localized tenderness and a decreased range of motion in the involved bone and nearby joint, muscle atrophy, a palpable mass, soft tissue swelling, and joint effusion in the affected area. Less commonly, pathological fractures can be found, especially in cases involving the foot. In cases involving the temporal bone, tinnitus, dizziness, and hearing loss have been reported.
In a publication by Turcotte et al. it was found that the average duration of symptoms for patients with chondroblastoma was about 20 months, ranging from 5 weeks to 16 years.
Chondroblastoma is a rare, benign, locally aggressive bone tumor that typically affects the epiphyses or apophyses of long bones. It is thought to arise from an outgrowth of immature cartilage cells (chondroblasts) from secondary ossification centers, originating from the epiphyseal plate or some remnant of it.
Chondroblastoma is very uncommon, accounting for only 1-2% of all bone tumors. It affects mostly children and young adults with most patients being in the second decade of life, or less than 20 years of age. Chondroblastoma shows a predilection towards the male sex, with a ratio of male to female patients of 2:1. The most commonly affected site is the femur, followed by the humerus and tibia. Less commonly affected sites include the talus and calcaneus of the foot and flat bones.
Giant-cell fibroblastoma is a rare type of soft-tissue tumor marked by painless nodules in the dermis (the inner layer of the two main layers of tissue that make up the skin) and subcutaneous (beneath the skin) tissue. These tumors may come back after surgery, but they do not spread to other parts of the body. They occur mostly in boys and are related to dermatofibrosarcoma protuberans.
Pilomatricomas consist of anucleate squamous cells (called "ghost cells"), benign viable squamous cells and foreign body giant cells. These neoplasms have a characteristic transition of cells. The lining of the cyst consists of basoloid cells with indistinct cell borders and basophilic nuclei that mature into the eosinophilic anucleated squamous cells. The presence of calcifications with foreign body giant cells is common with in the tumors.
Epulis (; plural "epulides") is any tumor like enlargement (i.e. lump) situated on the gingival or alveolar mucosa. The word literally means "(growth) on the gingiva", and describes only the location of the mass and has no further implications on the nature of the lesion. There are three types: fibromatous, ossifying and acanthomatous. The related term parulis (commonly called a gumboil) refers to a mass of inflamed granulation tissue at the opening of a draining sinus on the alveolus over (or near to) the root of an infected tooth. Another closely related term is gingival enlargement, which tends to be used where the enlargement is more generalized over the whole gingiva rather than a localized mass.
This type of epulis is neither pyogenic ("pus producing") nor a true granuloma, but it is a vascular lesion. About 75% of all pyogenic granulomas occur on the gingiva, although they may also occur elsewhere in the mouth or other parts of the body (where the term epulis is inappropriate). This common oral lesion is thought to be a reaction to irritation of the tissues and poor oral hygiene. It is more common in younger people and in females, and appears as a red-purple swelling and bleeds easily.
Jaffe–Campanacci syndrome is one of the disorders associated with café au lait macules (CALMs). Presentations may include Intellectual Disability, disseminated non-ossifying fibromas of the long bones and jaw, hypogonadism or cryptorchidism, or giant cell granulomas of the jaw.
It was characterized in 1958 and 1983.
A giant solitary trichoepithelioma is a cutaneous condition characterized by a skin lesion that may be up to several centimeters in diameter.