Home About us Contact | |||
Hamartoma
Kinds of Hamartoma Selected AbstractsHamartoma of the breastJOURNAL OF MEDICAL IMAGING AND RADIATION ONCOLOGY, Issue 2007A Murat SUMMARY Breast hamartoma is a rare benign tumour that leads to unilateral breast enlargement without a palpable localized mass lesion. Histologically, a hamartoma consists of varying amounts of adipose, gland, fibre and smooth muscle tissue. The characteristic mammographic appearance of hamartoma of breast has distinct mammographic features with circumscription and fat and soft-tissue density surrounded by a thin radiopaque capsule or pseudocapsule. Ultrasonographic findings include a well-defined mass lesion consisting of echogenic and sonolucent areas. We present histopathological and radiological findings of a 42-year-old female patient with breast hamartoma who has no complaint. [source] Precalcaneal Congenital Fibrolipomatous Hamartoma: A Report of Four CasesPEDIATRIC DERMATOLOGY, Issue 6 2000Carmen Ortega-Monzó M.D. The lesions were asymptomatic, bilateral, and symmetric, and measured approximately 1 cm in diameter. Their size increased in proportion to the growth of the child. In three infants the lesions persisted at 1 year of age, while in the fourth they remained at age 12 years. The nodules were not associated with any other disease or abnormality. A review of the literature revealed only one Argentinean series comprising four cases, and two communications at international pediatric congresses by French and American authors who reported five and three cases, respectively. We describe four additional cases of precalcaneal congenital fibrolipomatous hamartoma and comment on their histopathologic and ultrastructural characteristics. [source] Hamartoma of the Breast in a Man: First Case ReportTHE BREAST JOURNAL, Issue 4 2001Keyvan Ravakhah MD Abstract: Mammary hamartomas were reported in 0.7% of all benign tumors of the female breast. Histologically breast hamartomas contain lobular breast tissue with various degrees of fibrous, fibrocystic, and adipose tissue. Rare types include muscular (myoid) and cartilage (chondroid) hamartomas. We report a case of muscular hamartoma in a man. A 36-year-old man was admitted to the psychiatric unit with the diagnosis of schizophrenia. The patient complained of a slowly growing mass in his left breast. He denied any discharge from the nipple, but he complained of itching. A 2 cm × 3 cm nontender mass was palpable. There was no evidence of axillary lymphadenopathy. A needle aspiration was nondiagnostic. The excisional biopsy specimen revealed fatty tissue which was edematous and hemorrhagic. Microscopically it showed multiple bundles of muscles organized randomly. Myoid hamartoma was the diagnosis. Mammary hamartoma is considered a female tumor exclusively. Myoid hamartoma has been reported previously in 25 women. We report a myoid hamartoma in a man and, to our knowledge, it is the first and only such case to be reported. [source] Incidence and cytological features of pulmonary hamartomas indeterminate on CT scanCYTOPATHOLOGY, Issue 3 2008A. Saqi Objective:, Pulmonary hamartomas have a characteristic heterogeneous radiological appearance. However, when composed predominantly of undifferentiated mesenchymal fibromyxoid component, their homogeneous appearance on computed tomography is indeterminate for malignancy. Rendering an accurate preoperative diagnosis in these cases can alter management. The aim of this study was to determine the incidence and accuracy of cytodiagnosis for hamartomas ,indeterminate' by imaging. Methods:, We retrospectively reviewed records for hamartomas diagnosed by transthoracic fine needle aspiration (FNA) including immediate impressions and final diagnoses. Cytological features evaluated included the presence of fibromyxoid stroma, bronchioloalveolar cell hyperplasia, fibroadipose tissue, cartilage and smooth muscle. Results:, Eighteen (1.3%) hamartomas were identified from 1355 transthoracic FNAs. The immediate impression was hamartoma in 13 (72%), carcinoid in one (6%), mucinous bronchioloalveolar carcinoma in two (11%) and non-diagnostic in two (11%). The final diagnosis of hamartoma in cases diagnosed as carcinoid, mucinous bronchioloalaveolar carcinoma and non-diagnostic on immediate impression was rendered following assessment of all cytological material. Conclusion:, Overall, FNAs are highly reliable for diagnosing hamartomas even when composed principally of undifferentiated mesenchymal fibromyxoid stroma, especially with the aid of all available preparations including Diff-Quik smears, Papanicolaou smears, ThinPreps and cell block material. [source] Sebaceous Carcinoma, Basal Cell Carcinoma, Trichoadenoma, Trichoblastoma, and Syringocystadenoma Papilliferum Arising within a Nevus SebaceusDERMATOLOGIC SURGERY, Issue 12p2 2004Christopher J. Miller MD Background. Nevus sebaceus has a well-documented potential to develop a wide variety of neoplasms of both epidermal and adnexal origins. It is highly unusual for more than three tumors to arise simultaneously within a single nevus sebaceus. Sebaceous carcinoma arising within a nevus sebaceus is a rare occurrence. Objective. The objective was to report the case of a patient with a nevus sebaceus that simultaneously developed five distinct neoplasms of epidermal and various adnexal origins and to report the fourth case of sebaceous carcinoma arising within a nevus sebaceus. Methods. A 45-year-old woman presented with a nevus sebaceusthat contained five separate neoplasms, including sebaceous carcinoma, basal cell carcinoma, trichoadenoma, trichoblastoma, and syringocystadenoma papilliferum. Results. Complete excision of the nevus sebaceus and the five tumors was performed. Systemic work-up showed no evidence of metastatic disease or association with Muir-Torre syndrome. Conclusion. This case report highlights the diverse neoplastic potential of nevus sebaceus and demonstrates the capacity of this hamartoma to develop aggressive tumors, such as sebaceous carcinoma. Prophylactic excision or at least close clinical surveillance for sudden development of new growths is warranted in all cases of nevus sebaceus. [source] Different Electroclinical Manifestations of the Epilepsy Associated with Hamartomas Connecting to the Middle or Posterior HypothalamusEPILEPSIA, Issue 9 2003Alberto J. R. Leal Summary:,Purpose: The epilepsy associated with hypothalamic hamartomas (HHs) has typical clinical, electrophysiologic, and behavioral manifestations refractory to drug therapy and with unfavorable evolution. It is well known that only sessile lesions produce epilepsy, but no correlation has been established between the different types of sessile hamartomas and the diverse manifestations of the epilepsy. We correlate anatomic details of the hamartoma and the clinical and neurophysiologic manifestations of the associated epilepsy. Methods: HHs of seven patients with epilepsy (ages 2, 25 years) were classified as to lateralization and connection to the anteroposterior axis of the hypothalamus by using high-resolution brain magnetic resonance imaging. We correlated the anatomic classification with the clinical and neurophysiologic manifestations of the epilepsy as evaluated in long-term (24 h) video-EEG recordings. Results: HHs ranged in size from 0.4 to 2.6 cc, with complete lateralization in six of seven patients. Ictal manifestations showed good correlation with the lobar involvement of ictal/interictal EEGs. These manifestations suggest the existence of two types of cortical involvement, one associated with the temporal lobe, produced by hamartomas connected to the posterior hypothalamus (mamillary bodies), and the other associated with the frontal lobe, seen in lesions connecting to the middle hypothalamus. Conclusions: A consistent clinical and neurophysiologic pattern of either temporal or frontal lobe cortical secondary involvement was found in the patients of our series. It depends on whether the hamartoma connects to the mamillary bodies (temporal lobe cases) or whether it connects to the medial hypothalamus (frontal lobe cases). [source] Congenital smooth muscle hamartoma on the scalpINTERNATIONAL JOURNAL OF DERMATOLOGY, Issue 6 2009Ayla Günlemez MD No abstract is available for this article. [source] Extensive inflammatory nevus comedonicus involving half of the bodyINTERNATIONAL JOURNAL OF DERMATOLOGY, Issue 6 2004Necmettin Kirtak MD Nevus comedonicus is an uncommon variant of adnexal hamartoma without known cause. It usually occurs on the face, neck and chest and appears as groups of closely arranged dilated follicular openings with keratin plugs. We report extensive inflammatory nevus comedonicus in a 14-year-old boy involving half of his body, limited by the midline, with suppuration and residual scarring. The lesions first appeared at 3 years of age and worsened at the start of puberty. The clinical features, etiopathology, histopathology and treatment options of nevus comedonicus are briefly reviewed in the light of the literature. [source] Folliculosebaceous cystic hamartoma of the nipple: a case reportJOURNAL OF CUTANEOUS PATHOLOGY, Issue 5 2009AbdullGaffar Badr Folliculosebaceous cystic hamartoma (FCH) is a relatively rare and recently described lesion of the skin. Mostly, it involves the skin of the head and neck area. It is considered by most authors as a form of non-neoplastic cutaneous hamartoma comprising follicular, sebaceous and stromal elements. However, few regard it as a sebaceous trichofolliculoma, i.e. a variant of trichofolliculoma. FCH of the nipple of female breast is a very rare lesion with only one previous case reported so far in the literature. We report a similar case with a brief review of the literature. [source] Clinicopathological and immnuohistochemical findings in a series of folliculosebaceous cystic hamartomaJOURNAL OF CUTANEOUS PATHOLOGY, Issue 2 2009Jose M. Suarez-Peñaranda Background:, Folliculo-sebaceous cystic hamartoma (FSCH) is an uncommon skin condition presenting as a slow-growing papulo-nodular lesion, in or around the nose. Most cases are not clinically suspected and only histopathological examination allows the diagnosis. Pathological features include a dermal-located infundibulo-cystic structure with sebaceous glands radiating around, a stromal component encircling the epithelial structures, with clefts between the lesional epithelial and stromal parts, as well as between this and the adjacent dermis. Results:, We report eight patients with the diagnosis of FSCH (5 females and 3 males), with ages ranging from 35 to 77 years. Most cases (5 out of 8) were located in or around the nose and sizes were comprised between 0.6 and 1.2 cm. Lesions had grown for long periods of time, up to ten years in one case. Immunohistochemistry showed staining for p63 in the epithelial component of all lesions, while CD10 was only present in some sebocytes. CD34 and Factor XIIIa positive cells were present in the lesional stroma. Staining for androgen and alpha-estrogen receptors was also usually noticed. Conclusions:, FCSH is a hamartomatous skin lesion, clinically indistinct but with well-defined histopathological features. Immunohistochemistry shows a profile very close to normal sebaceous glands. [source] Multiple mucinous and lipomatous variant of eccrine angiomatous hamartoma associated with spindle cell hemangioma: a novel collision tumor?JOURNAL OF CUTANEOUS PATHOLOGY, Issue 4 2006Hae-Woong Lee In most cases, EAH arises as a single lesion; however, multiple variants have been reported. We report a 35-year-old female patient with multiple, sudoriparous, subcutaneous nodules on the right foot, which showed typical histopathological findings of EAH, and vascular components of the tumor consisted of thin-walled dilated vascular spaces intermixed with spindle cells and some histiocytoid endothelial cells representing spindle cell hemangioma (SCH). To our knowledge, the co-existence of EAH with SCH is a novel finding and not yet described. [source] Cutaneous mesenchymal hamartoma with mixed myogenous differentiationJOURNAL OF CUTANEOUS PATHOLOGY, Issue 4 2006John Edwin Schrecengost A 3-month-old infant girl presented with a polypoid lesion in the perianal region. No changes in this lesion had been noted since birth. Microscopic examination of the excised specimen showed a mixture of mesenchymal elements, dominated by haphazard thin fascicles of skeletal muscle. Collagen and vascular changes were also apparent. Immunohistochemistry showed positive staining for muscle-specific actin and desmin in the fascicular components of the lesion, and smooth muscle actin, desmin, and h-caldesmon positivity in a haphazard collection of muscle fibers in the deep dermis and anal submucosa. Numerous Verhoeff-van Gieson positive elastic fibers were also noted. Hamartomas containing skeletal muscle have rarely been reported outside of the head and neck region. They must be distinguished from a variety of other tumors, including juvenile rhabdomyoma, benign Triton tumor, and rhabdomyosarcoma. [source] Igloo-like prepuce: a peculiar aspect of smooth-muscle hamartoma of the genitalia?JOURNAL OF CUTANEOUS PATHOLOGY, Issue 2 2005Paolo Sbano Histopathological examination consisting of classical microscopy, immuno-histochemistry and electronmicroscopy of the surgical material provided insights into the etiopathogenesis of this rare disease. [source] Hamartoma of the breastJOURNAL OF MEDICAL IMAGING AND RADIATION ONCOLOGY, Issue 2007A Murat SUMMARY Breast hamartoma is a rare benign tumour that leads to unilateral breast enlargement without a palpable localized mass lesion. Histologically, a hamartoma consists of varying amounts of adipose, gland, fibre and smooth muscle tissue. The characteristic mammographic appearance of hamartoma of breast has distinct mammographic features with circumscription and fat and soft-tissue density surrounded by a thin radiopaque capsule or pseudocapsule. Ultrasonographic findings include a well-defined mass lesion consisting of echogenic and sonolucent areas. We present histopathological and radiological findings of a 42-year-old female patient with breast hamartoma who has no complaint. [source] Acquired cutaneous smooth muscle hamartomaJOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY & VENEREOLOGY, Issue 1 2005A Morales-Callaghan [source] Lipomatosis of the sciatic nerve: Report of a case and review of the literatureMICROSURGERY, Issue 1 2009Emmanouil M. Fandridis M.D. We report a case of lipomatosis of the sciatic nerve, also known as lipofibromatous hamartoma. A male, 26-year-old, presented with gait impairment, leg pain, and foot drop compatible with sciatic nerve's compression. The preoperative magnetic resonance imaging (MRI) revealed diffuse thickening of the proximal third of the sciatic nerve with adipose tissue interspersed among the nerve fascicles. Internal neurolysis was performed with microsurgical techniques under high magnification. The patient was followed up for a period of 34 months. He significantly recovered, and there was no clinical recurrence of the tumor. © 2008 Wiley-Liss, Inc. Microsurgery, 2009. [source] Cowden's syndrome (multiple hamartoma and neoplasia syndrome): diagnostic dilemmas in three casesORAL DISEASES, Issue 4 2000SI Chaudhry Cowden's syndrome is a multisystem disease inherited as an autosomal dominant trait with incomplete penetrance and variable expression. The disease has typical oral manifestations which often precede more systemic involvement, and the dental professional is therefore well placed to institute a regime of regular checks to ensure early treatment of any neoplasms which may occur. However, since not all of the classical signs are present in all patients, diagnosis may be difficult. The case report of a patient with most of the features of Cowden's syndrome is presented and features compared with two other possible cases. [source] Angiomyomatous hamartoma and associated stromal lesions in the right inguinal lymph node: A case reportPATHOLOGY INTERNATIONAL, Issue 8 2000Yoichi Sakurai Angiomyomatous hamartoma is a rare disease with a predisposition for the inguinal lymph nodes. A 51-year-old male patient visited a local hospital because of a right inguinal mass, measuring 3 × 4 cm in size, which was resected. The resected specimen showed irregularly distributed thick-walled vessels in the hilum, extending into the medulla and focally into the cortex of the node, eventually becoming more dispersed and associated with smooth muscle cells splaying into sclerotic stroma. These findings are compatible with an angiomyomatous hamartoma. Another tumor-like mass appeared shortly after the resection at the same location, but was not an angiomyomatous hamartoma, rather it was composed of edematous stromal tissue with proliferating smooth muscle cells. The stromal component included thick-walled blood vessels and lymphatics. Although it could not be determined whether these associated changes in the surrounding stroma are a cause or an effect of angiomyomatous hamartoma, they indicate the clinical difficulty in determining an appropriate area of resection and may provide clues to the pathogenesis of angiomyomatous hamartoma. [source] Precalcaneal Congenital Fibrolipomatous Hamartoma: A Report of Four CasesPEDIATRIC DERMATOLOGY, Issue 6 2000Carmen Ortega-Monzó M.D. The lesions were asymptomatic, bilateral, and symmetric, and measured approximately 1 cm in diameter. Their size increased in proportion to the growth of the child. In three infants the lesions persisted at 1 year of age, while in the fourth they remained at age 12 years. The nodules were not associated with any other disease or abnormality. A review of the literature revealed only one Argentinean series comprising four cases, and two communications at international pediatric congresses by French and American authors who reported five and three cases, respectively. We describe four additional cases of precalcaneal congenital fibrolipomatous hamartoma and comment on their histopathologic and ultrastructural characteristics. [source] Unusual Vaginal Angiomatous Neoformation in a 3-year Old PugREPRODUCTION IN DOMESTIC ANIMALS, Issue 2 2008M Beccaglia Contents A 3-year-old female pug (8 kg bodyweight) was referred by a local veterinarian for evaluation of vaginal bleeding. Historically, the owner indicated chronic haematic vaginal discharge initiated approximately 3 months ago, overall good appetite and slight general dullness. Two months prior to the visit, the bitch had been spayed by the local veterinarian, but vaginal bleeding persisted after surgery. Following a general examination and complete blood analysis, a genital tract examination was performed and, through vaginal endoscopy, a red, smooth, and apparently broad-based vaginal mass was found. An episiotomy was performed and on surgical exploration, the bleeding site was determined to be on the surface of a mass composed of two congested, adjacent cylindrical structures, symmetric to the vaginal sagittal midline, broad-based and quite regular on the surface. The mass appeared to be well delimited from the surrounding tissue by a thin capsula, and each cylindrical structure had a tributary vessel. Ectopic and dysplastic corpora cavernosa of the clitoris characterized by angiomatous proliferation, cavernous haemangioma and vascular hamartoma were considered in the differential diagnosis. The presence of small intralesional nerves identified by S100 immunostaining was used as a diagnostic clue to classify this unusual vaginal angiomatous neoformation as vascular hamartoma. [source] Multifocal mesenchymal hamartoma of the chest wallRESPIROLOGY, Issue 3 2006Semsi ALTANER Abstract: Chest wall hamartomas are extremely rare. Frequently mesenchymal hamartomas are presented as a single mass and contain some primitive mesenchymal elements such as chondroid and trabecular bone structures. A 60-year-old man presented to hospital with chest pain. Thirteen years earlier, his CXR and thoracic CT showed three masses on the right and two masses on the left, but he had not received any treatment thereafter. His CT showed the same masses present 13 years earlier, but they were bigger and right thoracotomy was undertaken. At thoracotomy, two sections of the mass in the right posterior mediastinum and one section of the mass in the right apex were excised. They had an occasional bloody appearance and contained small cystic areas, and some areas were extremely hard. Microscopic examination showed that the lesions consisted of mature adipose tissue, a large number of veins of different diameters and collagen tissue. Besides, primitive mesenchymal elements, lymphoid cell accumulations and trabecular bone structures were seen focally. Bilateral chest wall hamartomas are extremely rare and may be confused with malignancy. [source] Button osteoma: Its etiology and pathophysiologyAMERICAN JOURNAL OF PHYSICAL ANTHROPOLOGY, Issue 3 2002Vered Eshed Abstract The present study investigates a circumscribed bony overgrowth on the cranial vault, known as button osteoma (BtO) and referred to here as button lesion (BtL). We discuss its anthropological implications. Data on its histology, location, and population distribution (by age, race, and gender) are provided. Microscopically, BtL is composed of well-organized dense lamellated bone which is poorly vascularized and with very few osteocytes. It forms a dome-shaped roof over an underlying diploeized area which includes the ectocranial table. The frequency of BtL is similar in modern (37.6%) and archaeological (41.1%) populations, in blacks, whites, males, and females, and correlates with age. It is rare in nonhuman primates. Fifty-five percent of the human skulls studied by us had BtL only on the parietal, 23.6% on the frontal, and 3.6% on the occipital bones. Fifteen percent had BtL on both the frontal and parietal bones. No lateral preference was found. Most skulls with BtL (64.1%) had only one lesion, 20.4% had two BtL, and 15.4% demonstrated multiple BtL. The average number of button osteomas on an affected skull was 1.97. The frequency of large osteomas (0.5,1.0 cm) was similar in young and old age groups. The demographic characteristics of BtL, mainly its high frequency among ancient and modern populations, its independence of sex and race, its scarcity in other primates, and the fact that its macro- and microstruture are indicative of an hamartoma (and not an osteoma or exostosis) suggest an evolutionary background to the phenomenon. Am J Phys Anthropol 118:217,230, 2002. © 2002 Wiley-Liss, Inc. [source] Hamartoma of the Breast in a Man: First Case ReportTHE BREAST JOURNAL, Issue 4 2001Keyvan Ravakhah MD Abstract: Mammary hamartomas were reported in 0.7% of all benign tumors of the female breast. Histologically breast hamartomas contain lobular breast tissue with various degrees of fibrous, fibrocystic, and adipose tissue. Rare types include muscular (myoid) and cartilage (chondroid) hamartomas. We report a case of muscular hamartoma in a man. A 36-year-old man was admitted to the psychiatric unit with the diagnosis of schizophrenia. The patient complained of a slowly growing mass in his left breast. He denied any discharge from the nipple, but he complained of itching. A 2 cm × 3 cm nontender mass was palpable. There was no evidence of axillary lymphadenopathy. A needle aspiration was nondiagnostic. The excisional biopsy specimen revealed fatty tissue which was edematous and hemorrhagic. Microscopically it showed multiple bundles of muscles organized randomly. Myoid hamartoma was the diagnosis. Mammary hamartoma is considered a female tumor exclusively. Myoid hamartoma has been reported previously in 25 women. We report a myoid hamartoma in a man and, to our knowledge, it is the first and only such case to be reported. [source] Surgical management of benign duodenal tumours,ANZ JOURNAL OF SURGERY, Issue 7-8 2010Ji-Qi Yan Abstract Background:, While benign duodenal tumours are rare compared with malignant tumours, they comprise a wide variety of pathologies. Despite their diagnostic challenge, the optimal management of benign duodenal tumours remains undefined. We aimed to review the diagnosis and surgical treatment of benign duodenal tumours. Methods:, Records of all patients with post-operative pathological diagnosis of benign duodenal tumour were retrieved. Information on clinical presentations, diagnostic methods, tumour locations, surgical approaches, pathological results and patient outcomes were analysed. Results:, The operative spectrum included local resection in 8 cases, segmental duodenectomy in 1 case, subtotal gastrectomy in 1 case, papilla resection with sphincteroplasty in 3 cases and pancreaticoduodenectomy in 5 cases. The post-operative pathology results indicated 5 cases of adenoma, 2 cases of tubular adenoma, 2 cases of villous adenoma, 2 cases of tubulovillous adenoma, 2 cases of hamartoma and 1 case each of hamartomatous polyp, Brunner's adenoma, adenomyoma, fibromatosis and ectopic pancreas. Post-operatively, one patient died of unrelated disease, one case was lost in follow-up and the remaining patients survived recurrence-free with a good quality of life. Conclusion:, The presentation of benign duodenal tumours is non-specific, with upper abdominal discomfort and upper gastrointestinal bleeding as common symptoms. Surgical resection is the preferable therapeutic choice with satisfactory prognosis. [source] Laryngeal hamartoma causing polyhydramnios and lethal pulmonary hypoplasiaAUSTRALIAN AND NEW ZEALAND JOURNAL OF OBSTETRICS AND GYNAECOLOGY, Issue 3 2010S. WEBB No abstract is available for this article. [source] Surgical removal of a localised vascular hepatic hamartoma in a dogAUSTRALIAN VETERINARY JOURNAL, Issue 9 2009M Gualtieri A 2-year-old male Labrador Retriever was presented for sudden abdominal distension. Abdominal ultrasonography revealed abundant abdominal fluid and a hepatic mass, which was removed by total lobectomy. Histologic evaluation of the mass supported a vascular hepatic hamartoma. Vascular hamartomas are rare malformations in animals and only two cases have been reported in the dog. This is the first recorded case of a canine vascular hepatic hamartoma treated surgically and having a successful long-term outcome. [source] Gingival vascular hamartoma in two calvesAUSTRALIAN VETERINARY JOURNAL, Issue 3 2004I. YERUHAM No abstract is available for this article. [source] Folliculosebaceous cystic hamartoma differentiates toward the infundibulum, sebaceous duct and sebaceous cells: immunohistochemical study of keratins and filaggrinBRITISH JOURNAL OF DERMATOLOGY, Issue 2 2009Y. Senba No abstract is available for this article. [source] Medallion-like dermal dendrocyte hamartoma: the main diagnostic pitfall is congenital atrophic dermatofibrosarcomaBRITISH JOURNAL OF DERMATOLOGY, Issue 1 2009M. Marque Summary Medallion-like dermal dendrocyte hamartoma is a newly described and rare clinical and pathological entity. This congenital, round, erythematous and atrophic lesion in the thoracic area is histologically characterized by a CD34+ dermal and hypodermal spindle-cell infiltration. We describe the clinical, histopathological, cytological and molecular features of three cases of dermal dendrocyte hamartoma. In all the cases, atrophic congenital dermatofibrosarcoma protuberans (DFSP) was the first histological diagnosis. In one case, wide surgery had been performed on the basis of the clinical and histological presentation. The histological pattern was similar in all the cases: epidermal atrophy and a spindle to ovoid cell proliferation in the dermis and in the subcutaneous fat. Immunochemical staining for CD34 and factor XIIIa was positive. Cytogenetic and molecular studies were performed; no chromosomal abnormality nor translocation t(17;22)(q22;q13) was observed. Fluorescence in situ hybridization analysis did not reveal the DFSP fusion gene COL1A1-PDGFB. We observed that the main diagnostic pitfall of medallion-like dermal dendrocyte hamartoma is atrophic congenital DFSP due to clinical and histological similarities. We emphasize that molecular studies to eliminate the t(17;22)(q22;q13) translocation of DFSP may provide determinant elements for diagnosis in order to avoid unnecessary mutilating surgery. [source] Onychoblastoma (hamartoma of the nail unit): a new entity?BRITISH JOURNAL OF DERMATOLOGY, Issue 5 2005C. Misciali No abstract is available for this article. [source] |