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Diffuse melanosis of the epithelial lining of an unusually large median raphe cyst of the penis is reported. Its light microscopic and ultrastructural features are described. The findings are discussed in the context of the published literature of genitourinary melanosis.
Colonic mucosal biopsies from patients with melanosis coli were examined by electron microscopy. They showed apoptosis of colonic surface epithelial cells and phagocytosis of resulting apoptotic bodies by intraepithelial macrophages. The latter migrated to the lamina propria where intracellular degradation of the apoptotic bodies resulted in formation of lipofuscin, characteristic of this condition. The results showed the pathogenesis of human melanosis coli was identical to that determined in an animal model of this disease.
Despite many studies on melanosis thyroidi, there is no consensus concerning the nature of the pigment or its pathogenesis. Here the past literature on this subject is reviewed and results are reported on a case of melanosis thyroidi that provides fresh insight into the nature of the pigment and its genesis. Briefly, the conclusion is that while a few of the pigment granules are classic lipofuscin granules (i.e., single-membrane-bound structures containing electron-dense material in the form of particles, granules and masses, and electron-lucent lipid droplets), a majority of the pigment granules also contain colloid, which is easily identified by its particulate substructure similar to the colloid in the lumen of follicles. Thus, these pigment granules are, in fact, ambilysosomes, where degradation of both endogenous (organelles) and exogenous (colloid) material occurs. Several phagosomes containing colloid were also seen in the thyroid epithelial cells.
Neurocutaneous melanosis is a rare congenital syndrome characterised by large or numerous congenital pigmented naevi and excessive proliferation of melanin-containing cells in the leptomeninges. The process is diffuse or multifocal, and has a tendency to infiltrate the neural tissue and the cerebrospinal cord; remote metastases may occur. There is usually histological evidence of malignancy (cellular pleomorphism and mitotic activity). Involvement of the basal cisterns is apt to cause internal hydrocephalus, and the prognosis is grave even when there is no histological evidence of malignancy. We present the case history and necropsy findings of a baby boy with neurocutaneous melanosis, followed by a brief review.
Two unusual cases of neurocutaneous melanosis are presented. Both patients had congenital giant hairy nevi and both developed hydrocephalus, seizures, and myelopathy. The first patient displayed multicentric cerebral and spinal cord melanosis, as opposed to the more commonly described basilar leptomeningeal involvement. The second patient had total spinal leptomeningeal involvement, and ventriculoperitoneal shunting for hydrocephalus produced peritoneal metastasis of melanoma. An individual born with a congenital giant hairy nevus or marked generalized cutaneous pigmentation should be closely observed for the development of malignant melanoma of the nervous system.
Neurocutaneous melanosis (NCM) is a rare congenital neurocutaneous syndrome characterized by large or multiple congenital melanocytic nevi and benign or malignant melanocytic tumors of the leptomeninges. The authors report the case of a 5-month-old girl with congenital giant melanocytic nevi who presented with symptomatic hydrocephalus. A right frontal ventriculostomy was performed in the patient. Magnetic resonance imaging demonstrated melanocyte accumulation within the hippocampi, medulla, and cerebellum. Cerebrospinal fluid cytology revealed no presence of melanocytes. A ventriculoperitoneal shunt was placed; the patient's neurological condition improved and she was discharged home in good condition. The diagnosis of neurocutaneous melanosis should be considered in a case in which an infant or child presents with hydrocephalus and either large or multiple (> or = 3) congenital melanocytic nevi. Although our patient's neurological status improved following treatment for hydrocephalus, there is no definitive therapy for NCM and symptomatic patients have a poor prognosis. Our case illustrates to the neurosurgeon the importance of recognizing the likelihood of underlying pathological conditions of the central nervous system in a child with cutaneous melanocytic nevi.
In excess of 300 Indian Robin (S. fulicata Lin.) males were examined during different phases of the reproductive cycle (180 during the breeding phase and 140 during the remaining phases) for the presence of melanin pigment in the testes. Melanosis (melanin localization) was evident during the regression (31%), preparatory (40%), and progressive (31%) phases in one or both testes of a specimen but was never observed in the breeding phase. Further, melanin was localized only in the intertubular area and was never found in the tubular elements or the tunic. Histomorphological studies of pigmented and nonpigmented testes and of the adrenals taken from these specimens suggest that spermatogenesis in the nonpigmented testes is slightly more advanced than in the pigmented testes and that the melanosis might have developed due to a malfunction of the adrenals.
We describe imaging findings in a 2-year-old girl with neurocutaneous melanosis and malignant cerebral melanoma. Because the cerebral melanoma in this child was of the amelanotic type, high-signal intensity on unenhanced T1-weighted images was not present. The cutaneous lesions played a crucial role in establishing a correct (presumed) histopathologic diagnosis on the basis of the imaging findings. To our knowledge this is the first report describing an intracranial amelanotic malignant melanoma in association with neurocutaneous melanosis.
We report on a patient developing simultaneous occurrence of lentigo maligna lesions, solar lentigines and an extensive melanosis of the oral mucosa. Diagnostically, epiluminescence microscopy had a relevant role in the preoperative assessment and selection of suspicious pigmented lesions, as the lesions histologically labelled as lentigo maligna and solar lentigo were clinically indistinguishable. We review the clinical, dermoscopic and histopathologic differential diagnosis of solar lentigo, malignant lentigo and mucosal melanosis with other melanocytic and keratinocytic lesions and discuss the possible relationship between these entities.
Melanosis peritonei is usually associated with benign cystic teratomas of the ovary. We describe a one-and-a-half-year-old girl with melanosis peritonei associated with enteric duplication cyst. Melanophages were seen in aggregates in and around the serosal blood vessels, nerve bundles, and scattered within the muscular wall of the cyst. Presence of hyperplastic nerve bundles associated with melanophages suggests their origin from the neural crest.
The authors describe the results of light- and electronmicroscopic investigation of the colonic mucosa in melanosis, occurring at a 35 years old male patient. In the tunica propria mucosae pigment storage macrophages described histologically can be recognized as well by the aid of electron microscope. In contrast to the light-microscopic picture, electronmicroscopically separation of the epithelial cells of the mucosa, accumulation of foreign material in the enlarged intercellular spaces, accumulation of granular-fibrillar matrix in the tunica propria and presence of plasma cells with increased secretory activity can be revealed. It is assumed, that separation of epithelial cells of the mucosa goes with the disturbance of the absorptive function. It can not be excluded, that electronmicroscopically revealed lesions of the coolnic musoca aggravate the severe obstipation. For this reason early diagnosis of the melanosis coli and ceasing the medication with athranol-glycosida-containing laxatives are very important.
Neurocutaneous melanosis is a rare congenital syndrome characterized by the association of large or multiple congenital melanocytic nevi and benign or malignant melanotic tumors in the central nervous system. Patients with neurocutaneous melanosis usually have neurological symptoms early in life that progress rapidly due to the development of increased intracranial pressure or malignant melanoma. We report a 2-month-old female infant with multiple congenital melanocytic nevi and frequent seizure attacks. Magnetic resonance imaging of the brain demonstrated several regions compatible with melanotic deposits. During follow-up for one year, she had normal development and was seizure-free under the treatment of phenobarbital and valproic acid. We suggest that infants with large or multiple congenital melanocytic nevi should receive regular clinical check-up and brain imaging to exclude the possibility of central nervous system lesions.
CASES: We present four cases of ocular melanosis. Choroidal melanoma was detected in all of them. Three eyes had decreased visual acuity and were enucleated because of their large, active tumours. In the fourth case the melanoma was detected in a routine examination and we were able to apply a preserving treatment with I125 brachytherapy. DISCUSSION: Melanosis oculi is often underestimated as a risk factor for uveal melanoma and glaucoma. Ophthalmic surveillance, every 6 or 12 months is important, in patients with ocular melanocytosis for early detection of high risk diseases.
It is widely known that long-term use of anthranoid-containing laxatives is the cause of melanosis coli. We describe a case of melanosis coli, which occurred in a 39-year-old liver transplant patient who took an over-the-counter product containing aloe, rheum and frangula. The typical brownish pigmentation of the colonic mucosa developed in a period of ten months. The anthranoid medication was stopped and follow-up colonoscopy one year later showed normal looking mucosa once more. However, in contrast to previous examinations, a sessile polypoid lesion was found in the transverse colon. Histology showed tubulovillous adenoma with extensive low-grade dysplasia. Since there have been preliminary reports suggesting a possible role of anthranoid-containing laxatives in the development of colorectal adenomas and cancer, their use should be discouraged.
A case of multiple giant congenital melanocytic naevi in whom central nervous system melanosis was detected at 6 weeks of age is described. The infant was asymptomatic, but presence of risk factors such as multiple naevi, giant naevi and naevi on scalp and posterior axial location prompted a magnetic resonance imaging study of the brain. To our knowledge, neurocutaneous melanosis at such a young age has not been reported in Indian literature.
The neurocutaneous melanosis (NCM) is a rare, neuroectodermal dysplasia defined by the association of giant or multiple, nonmalignant pigmented cutaneous nevi with leptomeningeal melanosis or melanoma. As a rule, the cerebral pathological substratum is characterized by a melanocytic infiltration of the leptomeninges, often leading to hydrocephalus. The most frequent clinical symptomatology starts early in life, with convulsive seizures, psychomotor delay, intracranial hyperpression: the prognosis is severe. Malignant melanomas can also occur. One 21 years-old patient affected by NCM with a giant bathing nevus and epilepsy is reported. Her psychomotor development was slightly delayed. Academic progress was disturbed by the frequency of seizures and the multiple dermatological surgeries, and she remained at the elementary school level. Her epilepsy appeared at seven years and became pharmacoresistant. It was a focal, left temporal epilepsy. Neuroimaging investigations were performed repeatedly, and demonstrated the progressive appearance of parenchymal lesions with T1 and T2 shortening, without contrast enhancement, at the pons (11 years), the two hippocampi (14 years), and of an atrophy of the cerebellum and the brainstem (19 years). No hydrocephalus, tumoral aspect, or meningeal involvement were demonstrated. This patient's case is peculiar because her neurological symptomatology consists only of focal epilepsy, unrelated to a tumor, with moderate cognitive impairment despite a rather long course of the disease. Her evolution raises the question of condidency to surgical treatment.
BACKGROUND: Melanosis of the uterine cervix is a rare, benign, melanocytic lesion of the cervix. A review of the English-language literature revealed 11 other reported cases. CASE: A 52-year-old African American woman who presented for evaluation and treatment of menorrhagia was found to have an irregular, pigmented cervical lesion. The ectocervical biopsy showed melanosis characterized by hyperkeratosis, acanthosis and prominent elongation of rete with abundant basilar pigmentation. Immunohistochemical staining showed scattered, basally situated, S-100 protein-positive melanocytes. CONCLUSION: The existence of this entity is further evidence of a spectrum of melanocytic lesions, including blue nevi and primary melanomas, known to occur within the cervix proper.
Intussusception of the appendix is a rare occurrence. Due to the similarity of its symptoms with appendicitis, preoperative diagnosis of this condition is extremely difficult. In this report, we present appendiceal intussusception with histological melanosis coli that occurred in a patient on long-term anthranoid laxative use for chronic constipation. Melanosis coli in the appendiceal tissue, as an indicator of chronic laxative intake, may be a clue implying that the appendical exposure to hyperperistalsis for a long time in our case led to the intussusception. We conclude that colonoscopy may help in preoperative diagnosis of appendiceal intussusception in patients with suspicious appendicitis, particularly in those using laxative medication.