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Trichoblastoma (TB) is a benign cutaneous adnexal neoplasm. The lesion typically presents as a slow-growing, solitary, well-circumscribed nodule measuring up to 3 cm in diameter. On rare occasions, TB causes malignant transformation into an aggressive form described as high-grade trichoblastic carcinoma. Four such cases have been reported to date; all were described as high-grade trichoblastic carcinomas. Here, we describe the case of a 72-year-old Japanese male patient with a rapidly enlarging subcutaneous tumor on his lower back, which was diagnosed as high-grade trichoblastic carcinoma. Histopathologically, the tumor featured both benign and malignant components, and a transition zone between these states was clearly evident. In the immunohistochemical analysis, a malignant component was positive for p53 and showed stronger staining of phospho-RAC-α serine/threonine-protein kinase (AKT) Ser473 in comparison with a benign component. These results suggest that loss of p53 function and activation of phosphatidylinositol 3-kinase-AKT signaling pathways played important pathogenic roles in malignant transformation of the present case., (© 2018 Japanese Dermatological Association.)

We report a case of microcystic adnexal carcinoma in a 80-year-old woman. This is a rare malignant adnexal cutaneous tumor with glandular and follicular differentiation, rare, often asymptomatic, with late diagnosis and slow growth, locally aggressive and rarely metastatic. The distinction with other benign and malignant skin tumors is difficult., (Copyright © 2018 Elsevier Masson SAS. All rights reserved.)

Background: Microcystic adnexal carcinoma (MAC) is a rare infiltrative cutaneous tumor, but with its locally aggressive nature, high rate of recurrence, and rare metastatic potential, it is an important clinical entity to consider when faced with a nipple lesion. These tumors are frequently misdiagnosed based on superficial biopsy alone., Case Report: This case of a 15-year-old girl with MAC of the nipple demonstrates the importance of having a high index of suspicion in order to obtain a diagnosis, to determine the extent of disease, and to achieve adequate resection before reconstruction. Close clinical observation is recommended due to the possibility of future recurrences., Conclusions: Between the confusing nomenclature, indolent course, and inadequacy of superficial biopsy to achieve an accurate diagnosis, MAC is a clinical entity that the plastic surgeon must be aware of in order to facilitate proper diagnosis and treatment.

Background: Adnexal carcinomas (ACs) are rare cutaneous malignancies of sweat gland or pilosebaceous origin. Optimal treatment and metastatic potential of AC are poorly defined. Mohs micrographic surgery (MMS) has been increasingly used to treat AC., Objective: To review selected primary cutaneous AC and their treatment outcomes with MMS., Materials and Methods: Literature review using PubMed search for articles related to primary cutaneous ACs., Results: Sebaceous carcinoma treated with MMS recurred and metastasized in 6.4% and 3.7%, respectively. Primary cutaneous mucinous carcinoma treated with MMS recurred and metastasized in 9.6% and 6.4%, respectively. After MMS, 4.7% of microcystic AC recurred with no reported metastases. After MMS, recurrences and metastases of trichilemmal carcinoma or hidradenocarcinoma have not been reported. Two (4.2%) regional lymph node metastases but no distant metastases or local recurrences have been reported in eccrine porocarcinoma. Squamoid eccrine duct tumor, pilomatrix carcinoma, and spiradenocarcinoma treated with MMS are also reviewed., Conclusion: The rarity of ACs and the lack of comparative data on treatment makes conclusive recommendations on treatment difficult. Recent large case series and reviews suggest MMS is a useful and possibly superior treatment for AC and should be considered if primary cutaneous disease is suspected.

Trichoblastic carcinoma is a rare epithelial malignant epithelial tumor, its diagnosis is difficult and the therapeutic management is non-consensual. This retrospective study, carried out between 2009 and 2015, covered 21 cases and analyzed the diagnostic and therapeutic characteristics of trichoblastic carcinomas. Sex ratio is 2. Trichoblastic carcinoma predominated in the face (65% of cases), particularly in perinasal (30% of cases). Its clinical presentation is in 95% of cases as basal cell carcinoma, which is the first clinical diagnosis evoked. The average size of the tumors was 2.3cm in diameter (from 0.7cm to 15cm). The treatment of these tumors is surgical: the margins retained were on average 0.7cm (0.5cm to 1cm). The first excision was mostly performed under local anesthesia, healthy borders were found in less than 40% of cases, requiring another intervention under general anesthesia with reconstruction by flap or skin graft in nearly 80% of cases. The lymph node metastasis rate was 5%. Three cases of recurrence (17%) occurred between 18 months and 6 years follow-up, despite complete resection. One case recurred three times. These results highlight the difficulty of diagnosing trichoblastic carcinomas, often confused with basal cell carcinomas. Though larger, poorly limited and infiltrating, trichoblastic carcinomas are not really distinguished from basal cell carcinomas. Only the anatomopathological examination of the excision piece make it possible to conclude, the biopsy being most often insufficient. Their local aggressiveness requires a greater margin of excision. The micrographic analysis of Mohs, for the periorificial lesions of the face, would reduce margins, increase their reliability and limit the number of surgical revisions. Finally, the literature reports a high rate of ganglion and visceral metastases (between 9.5 and 11%). Initial search for distal lymph node or metastatic involvement is essential, as well as regular clinical follow-up., (Copyright © 2017. Published by Elsevier Masson SAS.)

Microcystic adnexal carcinoma is a rare cutaneous tumor that is often misdiagnosed and has the potential to be aggressive. Mohs surgery is the treatment of choice to prevent recurrences. We present a case of a large recurrent microcystic adnexal carcinoma on the sternum, initially diagnosed as a basal cell carcinoma. This tumor infiltrated the muscle and bone and was unresectable with Mohs surgery.

J Drugs Dermatol. 2017;16(2):180-181.

Bowen's disease (BD)/carcinoma is a type of squamous cell carcinoma of the skin, however, the possibility of adnexal differentiation (the development of sebaceous carcinoma or porocarcinoma) occurring in BD/carcinoma has been suggested. We herein describe a case of BD with superficial invasive carcinoma, which showed a clear cell focus, demonstrating tricholemmal differentiation. This clear cell focus showed the following findings: primarily composed of clear cells, somewhat columnar clear cells aligned in a palisade along a discernible basement membrane, tricholemmal keratinization and glycogen contained within the cells. In addition, the immunohistochemical profile in this clear cell focus, namely, negative staining for cytokeratin (CK)1 and positive staining for CK17 and calretinin in the inner cells of the neoplastic lobule, corresponded to that of the outer root sheath cells. This case suggested that adnexal differentiation can rarely occur within true BD/carcinoma, although adnexal carcinomas are commonly associated with a simple bowenoid change., (© 2015 Japanese Dermatological Association.)

The microcystic adnexal carcinoma is a rare, locally aggressive malignant adnexal neoplasm associated with significant morbidity. It is often underdiagnosed due to clinical and histopathological resemblance with other cutaneous neoplasms and/or a combination of lack of familiarity associated with inadequate samples. We report a case with clinical hypothesis of scarring alopecia and histopathological diagnosis of microcystic adnexal carcinoma with favorable outcome in a follow-up of eleven years, after surgical treatment.

Objective: Trichoblastomas are rare and benign tumors that arise from rudimentary hair follicles. Presentation varies from superficial plaques to papular or nodular lesions. Trichoblastomas usually arise on the head or neck. A few cases of other vulvar trichoblastic tumors such as trichofibromas and trichoepitheliomas have been reported to this day, but no such report of vulvar trichoblastoma exists., Materials and Methods: We report the case of a 61-year-old woman who presented with a vulvar trichoblastoma. The woman presented with a lump of the labium majus. She was managed surgically, first by wide excision of the mass followed by a second surgery consisting of a partial vulvectomy. Because surgical margins were positive and there is a potential for malignant transformation, a third surgery was performed. The margins came back negative. A literature review on trichoblastomas was performed, including its potential for malignant transformation, management, and immunohistochemistry to differentiate it from basal cell carcinoma., Results: Trichoblastomas are benign tumors that have a potential for malignant transformation. These tumors can present aggressive characteristics. Differential diagnosis from basal cell carcinoma can be difficult but is facilitated using immunohistochemistry., Conclusions: This is the first case of vulvar trichoblastoma reported in the literature. Although it is a benign tumor, excision with negative margins is recommended because it can be difficult to distinguish from basal cell carcinoma and it has a potential for malignant transformation.

Microcystic adnexal carcinoma (MAC) is an uncommon adnexal neoplasm with a predilection for the head and neck. The tumor rarely metastasizes but is locally aggressive and commonly demonstrates perineural invasion. MAC occurs most often in older adults. This report describes a young woman with a MAC in her left axilla who required two stages of Mohs micrographic surgery followed by a wide local excision because of persistent perineural invasion in close proximity to the brachial plexus. Other cases presenting in the pediatric age group are discussed., (Published 2014. This article is a U.S. Government work and is in the public domain in the USA.)

Microcystic adnexal carcinoma (MAC) is an uncommon, locally aggressive, malignant cutaneous tumor with pilar and eccrine differentiation. Mohs micrographic surgery is the treatment of choice for this condition, but specific histological findings can complicate MAC removal and leave doubt as to whether the tumor has been completely removed. Here we describe the clinical and pathological characteristics of a case in which a patient with an MAC underwent multiple reexcisions because of the presence of benign subclinical syringomatous proliferations adjacent to the primary lesion. Our case raises awareness of syringomatous proliferation, a benign process histologically similar but behaviorally distinct from a primary MAC. This experience highlights the importance of continued communication between dermatopathologists and dermatologic surgeons in providing quality patient care.

Background: The trichilemmal carcinoma is a rare malignant neoplasm, whose origin lies in the annexes of the skin, because of its low prevalence often confused with other dermal tumors, the differential diagnosis is not easy and is usually made by exclusion; in theory, its behavior is slow with little tendency to send both regional lymph node metastasis and systemic. Due to the limited number of cases there is no consensus on the prognosis, although it is generally considered good. The aim of this report is to show a case that, contrary to previous reports, the clinical presentation is aggressive with large soft tissue tumor infiltration around the site of origin in a patient without risk factors for skin cancer., Clinical Case: Male patient, 65 years old with cytologic diagnosis of carcinoma in a preprarotideal facial tumor, characteristics at diagnosis were infiltration of the facial skin, ear, and parotid gland. Complete block resection was performed, radical parotiroidectomy and radical neck dissection; the soft tissue defect was covered with a pediculated flap. The evolution was satisfactory it follow-up short though. We evaluated the experience in the literature regarding the prognosis and treatment of these patients., Conclusion: Trichilemmal carcinoma can be fully invasive behavior prognosis is difficult to know and probably depends on the clinical stage at diagnosis.

Introduction: Adnexial carcinomas are a group of rare and aggressive malignant skin tumors. Management of these tumors is not well codified., Patients and Method: The authors present a retrospective and single-center study of six cases. Surgery was performed between November 2005 and February 2008., Result: Pathological study revealed two syringoideccrine carcinomas, one microcystic adnexal carcinoma, two tricholemmal carcinomas, and one extra-ocular sebaceous neoplasm. In five cases, complete excision was guided by extemporaneous examination. In five cases, reconstructive surgery involved loco-regional skin flaps: two Abbé flaps, one Webster flap, one lifting lip flap, one submental flap, and one LLL flap. One case of relapse occurred, at 40months. Several particular characteristics of these tumors are shown in this study. They often follow a non-specific and discrete clinical course, are deeply infiltrated with a perineural tropism in some forms, and are preferentially located in the mid-face. Risks of incomplete excision and relapse are high., Conclusion: Complete excision is absolutely necessary in the management of these tumors, especially if reconstructive surgery is performed. Extemporaneous examination was in our experience a good option, particularly in the case of large labial or eyelid excisions., (Copyright © 2012 Elsevier Masson SAS. All rights reserved.)

Sclerosing sweat duct carcinoma (SSDC) in the nose, a rare occurrence with no specific symptoms, is frequently confused with rhinophyma or other inflammatory disease. In this report, we describe a 64-year-old woman who presented with a mass on her nasal dorsum for 1 year. Initially, the tumor had been misdiagnosed as a rhinophyma and followed-up for 1 year. She was referred due to a persistent nasal and neck mass. Deep incisional biopsy was performed to produce sufficient tissue and the histopathologic results showed SSDC. We performed wide local excision and reconstruction using radial forearm musculocutaneous free flaps with a costal cartilage framework followed by bilateral neck dissection. SSDC is seldom recognized and is frequently clinically confused with benign syringoma prior to pathologic diagnosis. Wide and deep incisional biopsy is necessary for the correct diagnosis and successful treatment of SSDC., (Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.)

Merkel cell (primary cutaneous neuroendocrine) carcinoma is a rare neoplasm of the skin. Its occurrence has been reported in association with other cutaneous neoplasms (Bowen disease, squamous cell carcinoma) in cases regarded as collision tumors. It has recently been described in association with cysts of the follicle apparatus. We present a unique case of rapidly growing nodular tumor on the left forearm of an 84-year-old woman, which proved to be a Merkel cell carcinoma located within a cystic trichoblastoma. The malignant component located in the center of the lesion had typical histopathological and immunohistochemical features of Merkel cell carcinoma. It was surrounded by an epithelial proliferation, made of K17-positive basaloid cells, whose aspects where those of trichoblastoma in a retiform pattern. Both lesions were intertwined, suggesting that the Merkel cell carcinoma had developed within a previously existing trichoblastoma and that it derived from the follicular Merkel cells present in the trichoblastoma. The unique features of this case, together with the reported cases of Merkel cell carcinoma arising within follicular lesions, and the fact that numerous Merkel cells are normally localized in the adult hair follicle, further support the hypothesis of a histogenetic link between normal follicular Merkel cells and Merkel cell carcinoma.

Cylindromatosis describes a rare autosomal dominantly inherited disease characterized by multiple cylindromas, which are located on the scalp and the neck. We report on an 80-year-old patient with a long history of multiple asymptomatic, skin-colored tumors on head, neck and upper part of the body. Clinical and histopathologic criteria lead to the diagnosis of cylindromatosis. Development of cylindrocarcinoma has been reported, so one must choose on an individual basis between close follow-up and prophylactic excision.

Microcystic adenexal carcinoma is a rare, locally aggressive, malignant appendage tumor also known as sclerosing sweat duct carcinoma. Since widespread recognition of microcystic adenexal carcinoma as a distinct clinicopathologic entity, approximately 300 total cases have been reported in the literature, with only eight previous cases reported in children under the age of 18, with no reported cases in patients younger than 6 years old. Our patient is unique in that the lesion was present at birth, making this the youngest case of microcystic adenexal carcinoma reported., (© 2011 Wiley Periodicals, Inc.)

Introduction: Microcystic adnexal carcinoma (MAC) is a rare cutaneous tumour where the role of radiotherapy remains undefined. We contrast our institutional experience with current literature, define the local control rate and suggest a role for adjuvant radiotherapy in the treatment of this cancer., Methods: From 1992-2008, a retrospective review was undertaken for MAC treated with curative intent involving surgery and adjuvant radiotherapy at the Royal Brisbane Hospital and Mater Hospital Brisbane. Clinical, pathologic and treatment details as well as patterns of recurrence were analysed., Results: Fourteen cases of MAC received local excision and adjuvant radiotherapy. The median age was 71 years old, with nine of the cases in men. All cases occurred in the head and neck region with mean tumour size of 20.5 mm and mean depth of invasion of 9.9 mm. Peri-neural invasion occurred in 56% of cases and 69% had positive surgical margins. Various adjuvant radiotherapy schedules were used to treat the primary site resulting in a crude local control rate of 93%. Primary and nodal relapses were subsequently salvaged with further treatment., Conclusion: While Mohs micrographic surgery may be considered the gold standard, wide local excision and adjuvant radiotherapy offers comparable control rates. Doses of 50 Gy or greater should be prescribed with generous margins (3-5 cm) owing to its tendency for peri-neural and deep invasion. There was no evidence that radiotherapy can cause aggressive transformation of the tumour. The role for definitive radiotherapy remains uncertain., (© 2010 The Authors. Journal of Medical Imaging and Radiation Oncology © 2010 The Royal Australian and New Zealand College of Radiologists No claim to original US government works.)

We report a very rare case of trichilemmal carcinoma (TLC) involving the upper eyelid. To the best of our knowledge, this is the first report of trichilemmal carcinoma of the upper eyelid in Korea. A 51-year-old man presented to our hospital complaining of a bloody discharge from his left upper eyelid. He had a soft and lobulated mass on the palpebral conjunctiva. An incisional biopsy revealed trabecular growth of tumor cells with clear cytoplasm, prominent nucleoli, frequent mitoses, and foci of trichilemmal keratinization. Immunohistochemically, the lesion was positive for p53 and negative for CD 34. A diagnosis of TLC was made, and total excision of the mass and reconstruction of the eyelid were performed. Trichilemmal carcinoma is a rare malignant tumor, though it appears to be an indolent neoplasm with no metastatic potential. The treatment of choice for trichilemmal carcinoma of the eyelid is complete excision with tumor-free margins due to the locally invasive nature of the lesion.

Spiradenocylindrocarcinoma is a very rare malignant cutaneous neoplasm of the folliculosebaceous-apocrine unit. We report a case of this hybrid tumor in a 42-year-old woman. The tumor consisted of 2 circumscribed nodules with areas of cylindrocarcinoma and low-grade spiradenocarcinoma. In the overlapping areas, both spiradenomatous and cylindromatous features were observed. Expansion of the tumor beyond the fibrous pseudocapsule into the adjacent tissue was present. Furthermore, tumor cells were demonstrating mild to moderate pleomorphism and an increased mitotic index. p53 and ki-67 were among the positive immunohistochemical markers. A relatively small number of tumor cells expressed estrogen receptors. The aim of this study was to investigate the nature of this rare tumor of the skin appendages.

Invasive carcinoma in the axilla may arise from skin appendage glands or ectopic breast tissue or it may be a metastasis. Carcinomas of the skin adnexal glands and breast can be difficult to distinguish from each other as they often display the same patterns of growth. Tubular, cribriform, papillary, apocrine, mucinous, and adenoid cystic are histologic types of carcinoma seen in the breast and skin appendage glands. To our knowledge, secretory carcinoma, the most common form of mammary carcinoma in children, has not yet been described as a morphologic pattern of skin adnexal carcinoma, although we cannot exclude the possibility that such a case was reported with a different diagnosis. We report a case of a young girl with secretory carcinoma that seems to have arisen from skin appendage glands in the skin of the axilla in the absence of demonstrable ectopic breast tissue.

Skin adnexal neoplasms are rare tumors, and knowledge on their exact histological development and pathogenesis remains fragmented. Their diagnosis and management are frequently troublesome, especially for malignant tumors. We present a retrospective case series of malignant appendageal tumors of the face. Records of patients with histologically confirmed adnexal skin carcinoma managed at a single department during the period 1995 to 2004 were reviewed. Patient biodata, surgical management, and main outcome measures such as locoregional recurrence, distant metastasis, and disease-free and overall survival were recorded.Eleven patients were identified from record analysis. Lesions were mainly located at the eyelids and the nose. All patients were managed with wide local excision and restoration with local flaps. Two patients showed regional metastasis and underwent radical neck dissection. Nine of 11 patients were alive and free of local and/or regional disease on final follow-up. Two patients died of causes unrelated to the disease. Clinical and histological features, tumor biological behavior, diagnostic difficulties, and recommended management are discussed.In conclusion, adnexal skin tumors should be considered in the differential diagnosis of skin tumors, particularly in view of their more sinister prognosis compared with other nonmelanoma skin tumors. They are acknowledged to demand a more aggressive plan of surgical treatment.

Microcystic adnexal carcinoma (MAC) is an uncommon, locally aggressive tumor. It typically involves the upper lip of middle-aged adults, and in rare instances the scalp. We report a Japanese woman with a giant MAC on the scalp. Physical examination revealed a 110 mm x 120 mm induration on her parietal region. Microscopically, the tumor showed both pilar and sweat gland differentiation. Resection included the cranium; for reconstruction we used a titan mesh allograft and covered it with a free latissimus dorsi muscle flap and a mesh skin graft. Ours is the first case of a MAC measuring more than 100 cm2 arising on the scalp of an individual in the third decade of life.

We report a case of an extremely rare histologic combination of pilomatrix or pilomatrical carcinoma with admixed melanocytes within the same tumor mass. Pilomatrix carcinoma is a neoplasm of low-grade malignancy that is characterized by a tendency for recurrence but low risk of metastasis. A 77-year-old male presented with a nodule on the bridge of the nose that was excised. Histologically, it was typified by asymmetry and poor circumscription, the presence of several variably sized and shaped basaloid aggregations, and surface ulceration. The tumors were composed of pleomorphic basaloid cells with prominent nucleoli and frequent mitoses (some of which were atypical) accompanied by central areas with keratotic material, shadow cells, and foci of necrosis. In addition, intermingled with the pilomatrix carcinoma, several easily identified pigmented cells with dendritic processes were present singly and as small aggregates. There was no atypia associated with the melanocytic component. Immunohistochemistry revealed the CK14 to be positive mainly within the keratinizing and the squamous epithelial elements of the tumor. The melanocytic component was strongly immunoreactive for S100, melanoma cocktail (HMB45 and Melan-A), and microphthalmia transcription factor. Pilomatrix carcinoma with melanocytes should be distinguished from the conventional pilomatrixoma with pigmentation, melanocytic matricoma, melanoma, and pigmented basal cell carcinoma with matrical differentiation. Clinicians and pathologists should be aware of the occurrence of pilomatrix carcinoma with melanocytes because of its potential for diagnosis as melanoma. This peculiar lesion recapitulates the intimate relationship existing between matrical epithelium and melanocytes in the embryonal hair follicle or in the anagen stage of the hair cycle. It is possible that sun damage played a role in stimulating migration of melanocytes among matrical cells in this case.

Pilomatrical carcinoma is a rare tumor without a single pathognemonic feature that distinguishes it from the benign pilomatricoma. We report a 51-year-old man with HIV, hepatitis C, and pilomatrical carcinoma.

Sclerosing sweat duct carcinoma is a rare, locally aggressive adnexal tumor that frequently occurs on the face of middle-aged adults, invades deeply, and has a propensity to recur. We report a rare instance of sclerosing sweat duct carcinoma occurring in a 6-year-old African American child and review the literature of this infrequently observed neoplasm.

We report an unusual example of trichoepithelioma containing giant and multinucleated cells in the epithelial compartment. The patient was a 52-year-old woman who presented with a solitary nodule on the scalp measuring 2 cm of apparently long duration. The biopsy revealed a typical trichoepithelioma. The unusual feature was the presence in some epithelial nodules of large epithelial cells with hyperchromatic nuclei and no visible nucleoli that exceed 3 to 5 times the adjacent follicular germinative cells. Often, multinucleated cells were seen. Rare nodules were almost entirely composed of giant/multinucleated cells. The majority of the nodules containing giant cells were situated in the deeper portion of the neoplasm. No mitoses were seen in these giant cells. No enlarged or multinucleated cells were seen in the stroma. The neoplastic epithelial cells were diffusely reactive for bcl-2, including the giant and multinucleated cells. The proliferating rate was low; Ki-67 stained some giant cells. CD34 stained the stroma. We believe that the focal presence of pleomorphic giant cells in trichoepithelioma has no clinical or prognostic implications and does not denote its "malignant transformation".