Primary mucinous carcinoma (PMC) of the skin—a case report

Primary mucinous carcinoma of the skin is a rare malignant adnexal neoplasm. It was first described by Lennox et al. in 1952. It is uncertain whether it is of apocrine or eccrine origin (Wright and Font, Cancer 44:1757–1768, 2; Mendoza and Helwig, Arch Dermatol 103:68–78, 3). Showing morphological similarity to mucinous carcinoma of the breast, it may be difficult to distinguish it from a cutaneous metastatic deposit of mucinous adenocarcinoma from a visceral primary lesion. Hence, it deserves attention not only for its scarcity but also for the challenge in determining its precise diagnosis and hence, planning its management. We present a case of primary mucinous carcinoma of the skin.     

Microcystic Adnexal Carcinoma of the Breast: A Very Rare Breast Skin Tumor

BACKGROUND: Microcystic adnexal carcinoma is a rare, slowly progressing, malignant tumor of sweat gland origin. OBJECTIVE: A case of microcystic adnexal carcinoma of the breast with a history of 20 years is presented. METHODS: On initial examination, the ulcerative cutaneous mass was fixed to the underlying breast tissue and chest wall. Both the macroscopic appearance of the tumor and its location suggested a glandular breast carcinoma. However, an incisional biopsy revealed the nature of the tumor as microcystic adnexal carcinoma of the breast skin. Following the diagnosis, a wide excision of the tumor was carried out for the final treatment. RESULTS: To our knowledge this is the first case of microcystic adnexal carcinoma arising in the breast skin presented in the literature. CONCLUSION: This case demonstrates that microcystic adnexal carcinoma can occur on the breast skin and should be treated with wide excision due to its locally aggressive behavior.     

Microcystic Adnexal Carcinoma (MAC) of the Scalp with Extensive Pilar Differentiation

BACKGROUND: Microcystic adnexal carcinoma (MAC), or sclerosing sweat duct tumor, is an uncommon, locally aggressive tumor. It typically involves the upper lip or face of middle-aged adults and rarely involves the scalp. It is characterized histologically by both pilar and eccrine differentiation and is associated with frequent perineural invasion. MAC is frequently misdiagnosed because of its bland and asymptomatic clinical presentation. In addition, its defining histologic features may be missed with a superficial biopsy. OBJECTIVE: To describe a case of MAC in the scalp treated with Mohs surgery. We describe a case with extensive pilar differentiation and clear cell changes. In addition, the clinical and histologic characteristics of this rare neoplasm are reviewed. METHODS: Mohs micrographic surgery (MMS) was performed on this neoplasm. RESULTS: The tumor was successfully excised in one surgery (five stages) and is without evidence of recurrence at 18 months. CONCLUSION: We present a case of MAC of the scalp in a 64-year-old white man treated successfully with MMS.     

Metastatic Microcystic Adnexal Carcinoma in an Immunocompromised Patient

BACKGROUND: Microcystic adnexal carcinoma is an uncommon, locally aggressive cutaneous neoplasm. To date, there are only two reports of histologically proven lymph node involvement with this tumor. We describe a case of a patient with microcystic adnexal carcinoma who developed multiple local metastasis in transit with histologically proven lymph node involvement and was diagnosed with chronic lymphocytic leukemia. OBJECTIVE: To describe the details of our case and to review what is currently known about this tumor. METHODS: Mohs micrographic surgery was utilized for tumor removal. RESULTS: This patient developed multiple tumors of the scalp over the period of a 1 year which were histologically proven to be microcystic adnexal carcinoma. All tumors were noncontiguous and presented on the scalp. During the histologic analysis of the last tumor removed by Mohs micrographic surgery a lymph node was resected which revealed infiltrative microcystic adnexal carcinoma. CONCLUSIONS: We present the case of an immunocompromised patient treated for microcystic adnexal carcinoma with Mohs micrographic surgery who proceeded to develop local metastasis in transit.     

The effect of radiation therapy on microcystic adnexal carcinoma: a case report

BACKGROUND: Microcystic adnexal carcinoma (MAC) is a rare, locally aggressive tumor. Treatment for this neoplasm typically requires extensive local excision leading to morbidity. Therefore, the temptation to use alternative treatment options remains high. METHODS: We report one case of a nasal dorsum MAC treated with external beam radiation secondary to the patient's poor health status and preference. RESULTS: After initial dramatic clinical resolution, the tumor recurred in a clinically more extensive and histologically more aggressive form. CONCLUSION: On the basis of this case and several detailed in the literature, we therefore hypothesize that radiation therapy is not only an ineffective treatment for MAC, but evidence exists that this modality may induce conversion to a histologically and clinically less favorable neoplasm.     

Microcystic adnexal carcinoma

Summary Microcystic adnexal carcinoma is a rare malignant neoplasm, with a highly aggressive local behavior, usually appearing in the upper lip of adult patients, but may also occur elsewhere in the face. Most often the tumor affects middle-aged females, but it also occurs in men. In this article, a case of microcystic adnexal carcinoma located in the upper lip of a 45-year-old male is described, it presented as a solitary, flesh-colored, indurated and painless plaque. A wide excision of the lesion with margin control was performed; reconstruction was with local flaps. The patient is free of recurrence 18 months later.     

Mucoepidermoid carcinoma of the skin: a distinct entity from adenosquamous carcinoma: a case study with a review of the literature

Mucoepidermoid carcinoma (MEC) of the skin is an exceedingly rare but distinctive neoplasm with respect to its histopathologic features. It is similar if not identical in most respects to MEC of the salivary gland, a neoplasm whose prognosis is correlated with the pathologic grade. We report a case of MEC of the skin in a 79-year-old white woman who presented with an axillary mass. Beneath an unremarkable epidermis, a circumscribed, cystic neoplasm, unattached to the surface, was characterized by the presence of vague lobules of low-grade-appearing squamous cells accompanied by mucigenic and clear cells. A mucin stain highlighted the mucigenic cells and immunohistochemistry revealed pan-cytokeratin, cytokeratin 7, polyclonal carcinoembryonic antigen, and epithelial membrane antigen positivity. The cytokeratin 20 and gross cystic disease fluid protein were nonreactive. Inconsistency was encountered in the literature where some confusion existed as to whether MEC is synonymous with adenosquamous carcinoma of the skin. Elsewhere in the body, the latter tumor type is a squamous and gland-forming neoplasm with intermediate- to high-grade features rather than a tumor with mucigenic cells intermingled among intermediate and squamous cells. As in the case of MEC and adenosquamous carcinoma elsewhere in extracutaneous sites, we would propose that a pathologic distinction should be made in the skin for the sake of consistency and for prognostic purposes. Additionally, the immunophenotype of our case is similar to at least two other cases of cutaneous MEC, as well as MEC of the salivary gland, to support the hypothesis that this neoplasm is adnexal rather than epidermal in origin.     

Microcystic adnexal carcinoma: an unpredictable aggressive neoplasm

Microcystic adnexal carcinoma is a rare, locally aggressive cutaneous neoplasm that has been recently recognized as a distinct entity. Typically it appears as a papular, flesh-colored mass on the face of women patients. Because of its benign appearance, it is often present for months or years before diagnosis. Histologically, there is a paucity of mitotic figures and other criteria usually associated with aggressive lesions. In this article we discuss 3 cases of microcystic adnexal carcinoma, each presenting as a small, localized lesion but all requiring extensive surgical extirpation with margin control using the Mohs tissue resection technique.     

Endocrine Mucin Producing Sweat Gland Carcinoma with Metastasis to Parotid Gland: Not as Indolent as Perceived?

Endocrine mucin-producing sweat gland carcinoma (EMPSCG) is a rare, low-grade cutaneous adnexal neoplasm with evidence of neuroendocrine differentiation, predominantly involving the eyelids of elderly. It has a striking resemblance to solid papillary carcinoma of breast which similarly displays neuroendocrine features. EMPSGC is considered a precursor of cutaneous mucinous carcinoma, and the term “mucinous carcinoma” is also recommended for hybrid lesions which reveal an invasive mucinous component associated with EMPSGC. While local recurrences are well- documented in EMPSGC, metastases had not been encountered until very recently; two reports in the past year have described metastases from eyelid EMPSGC to the parotid gland after a prolonged interval from the primary presentation. We report the case of a 78-year-old male with eyelid EMPSGC metastatic to the parotid gland nine years after excision of the primary tumor, which had initially been diagnosed as a poorly differentiated carcinoma. Development of metastasis after a prolonged interval is similar to both the previously described cases, and emphasizes the need to reevaluate the stated indolent nature of this neoplasm. It also aims to draw attention of pathologists to this uncommon tumor of the eyelid which is often misdiagnosed on primary presentation.     

Clinical Course, Risk Factors, and Treatment of Microcystic Adnexal Carcinoma: A Short Series Report

BACKGROUND: Microcystic adnexal carcinoma (MAC) is a locally aggressive neoplasm of both eccrine and follicular differentiation; it commonly presents on the head and neck of white middle-aged to older patients. OBJECTIVE: To review the course, risk factors, and management of 10 patients with microcystic adnexal carcinoma (MAC) and to compare treatment outcome by either Mohs micrographic surgery or standard surgical excision. METHODS: Tumor registry databases were used to identify cases at Roswell Park Cancer Institute in Buffalo, New York. Fifteen cases that were coded as MAC were reviewed. Ten cases were subsequently confirmed to be MAC. From these cases, data were collected on patient demographics, tumor location and size, risk exposure, metastasis, treatment, and recurrence of tumors. RESULTS: The average age of the patients was 63.4, and females outnumbered males seven to three. Nine tumors were located on the head and neck area. Five patients had a history of radiation therapy, and two patients were sisters. The first-time procedure was Mohs micrographic surgery in four cases and surgical excision in six cases. Three patients in the surgical excision group needed a second procedure, two of whom thereafter underwent Mohs micrographic surgery. In the Mohs micrographic surgery group, none of the patients required a second procedure, and all of the patients were disease free at an average of 23.3 months. One recurrence was noted in the surgical excision group, and the average follow-up was 32 months. CONCLUSION: This report supports the use of Mohs micrographic surgery for tumor control with the least possible procedures. Risk factors appear to include radiation treatment, ultraviolet exposure, and possibly genetics.     

Multiply Recurrent Trichilemmal Carcinoma With Perineural Invasion and Cytokeratin 17 Positivity

BACKGROUND: Trichilemmal carcinoma is an uncommon cutaneous malignancy that is thought to be the malignant counterpart of the trichilemmoma. Despite histologic features such as pronounced cytologic atypia, trichilemmal carcinoma is often described as having a rather benign clinical course. Cases of tumor recurrence after therapy are uncommon, and tumor neurotropism has never been described. OBJECTIVE: A case of multiply recurrent trichilemmal carcinoma with perineural invasion is described. The outer root sheath differentiation of this neoplasm is confirmed with the use of novel antibodies directed toward cytokeratins that are expressed in this area of the hair follicle. METHODS: The trichilemmal carcinoma was excised using the Mohs surgical technique. Tissue obtained during the extirpation of the tumor was subjected to immunohistochemical staining for cytokeratin 15, cytokeratin 17, and c-erb-B2. RESULTS: Tumor neurotropism was noted. The trichilemmal carcinoma demonstrated abundant cytoplasmic staining for cytokeratin 17 and c-erb-B2. CONCLUSIONS: In distinction to previous reports, this case reveals that trichilemmal carcinoma can demonstrate significant biological aggression, as reflected by tumor neurotropism and by failure to respond to multiple surgical excisions. The purported outer root sheath differentiation of this neoplasm is confirmed with the use of novel immunohistochemical staining. This immunohistochemical staining may be useful in differentiating trichilemmal carcinoma from other clear cell neoplasms.     

右肺巨大腺泡细胞癌合并纵隔严重偏移一例

原发性肺腺泡细胞癌极其罕见,世界卫生组织肿瘤分类把腺泡细胞癌定义为一种向(浆液)腺泡细胞分化的恶性上皮源性肿瘤.它被认为是一种低度恶性的肿瘤.自从1972年Fechner首次报道以来,迄今总共不足30例文献报道.因其罕见故容易与其它肺部肿瘤混淆而误诊.我们报道了 1例合并纵隔偏移的原发性肺腺泡细胞癌,女,27岁,右肺巨...     

Clear cell odontogenic tumor— a new histologic variant with aggressive potential

This report delineates the clinical and laboratory findings for a previously unreported neoplasm of putative odontogenic origin. We describe the clinical, radiographic, operative, histologic, histochemical, and ultrastructural findings of the tumor in three women. All lesions were centrally located in the jaw bones, were expansile, and, except for mobile teeth and/or jaw enlargement, exhibited few symptoms. Radiographically, there was bone and tooth destruction resulting in poorly delineated margins. Microscopically, the tumors were nearly identical in that they consisted primarily of large sheets and islands of uniform vacuolated and clear cells without evidence of amyloid deposition, calcification, or glandular differentiation. One of the lesions recurred 1 1/2 years after removal without evidence of metastasis. The results, based only on these cases, suggest that this is a locally aggressive neoplasm of odontogenic origin. Casual microscopic examination of this rare neoplasm could result in misdiagnosis as a metastatic clear cell neoplasm, especially renal cell carcinoma.     

Clear cell odontogenic tumor--a new histologic variant with aggressive potential

This report delineates the clinical and laboratory findings for a previously unreported neoplasm of putative odontogenic origin. We describe the clinical, radiographic, operative, histologic, histochemical, and ultrastructural findings of the tumor in three women. All lesions were centrally located in the jaw bones, were expansile, and, except for mobile teeth and/or jaw enlargement, exhibited few symptoms. Radiographically, there was bone and tooth destruction resulting in poorly delineated margins. Microscopically, the tumors were nearly identical in that they consisted primarily of large sheets and islands of uniform vacuolated and clear cells without evidence of amyloid deposition, calcification, or glandular differentiation. One of the lesions recurred 1 1/2 years after removal without evidence of metastasis. The results, based only on these cases, suggest that this is a locally aggressive neoplasm of odontogenic origin. Casual microscopic examination of this rare neoplasm could result in misdiagnosis as a metastatic clear cell neoplasm, especially renal cell carcinoma.     

Adenosquamous carcinoma of prostate

A case is reported of metastatic adenosquamous carcinoma that developed in a patient one year after diagnosis of adenocarcinoma of the prostate by transurethral resection of the prostate (TURP). Prostatic origin of the neoplasm was proved by immunoperoxidase staining for prostatic acid phosphatase in the metastases as well as demonstration of both glandular and squamous differentiation in tumor within the prostate on repeat TURP. This change in tumor differentiation occurred despite the fact that the patient had received no estrogen or radiation. The metastases showed remarkable response when the patient later began diethylstilbestrol (DES) therapy.     

Sebaceous Neoplasms

Sebaceous skin tumors are classified into sebaceous adenoma, sebaceoma, and sebaceous carcinoma. An additional group of cystic sebaceous tumors indicate the Muir-Torre syndrome (MTS). Cystic sebaceous tumors are considered as morphologic variants of the 3 main categories. Multilineage adnexal tumors with partly sebaceous differentiation may pose a challenge to categorize. Sebaceous hyperplasia and nevus sebaceus are not considered as true sebaceous tumor entities. Recently, attention has been drawn to morphologic clues of sebaceous differentiation. Immunohistochemistry using the mismatch repair proteins and/or genetic microsatellite instability testing should be performed on sebaceous neoplasms to diagnose MTS as early as possible.     

Mucin-producing Malignant Tumor of Lower Eyelid Presenting in a 14-year-old Patient

Malignancies of the ocular adnexa are rare, aggressive tumors with significant potential for local recurrence and metastases. Although basal cell carcinoma remains the most common malignancy of the eyelid, encompassing more than 90 percent of all periocular cancers, several other malignant neoplasms have been reported. Malignant ocular adnexal neoplasms are most commonly of sweat gland origin and include hidradenocarcinoma, mucinous eccrine adenocarcinoma, and apocrine adenocarcinoma of the glands of Moll. The different ocular adnexal adenocarcinomas share many of the same characteristics with regard to incidence, primary location, and rates of recurrence and metastases. As a result, these tumors are difficult to distinguish clinically and true diagnosis depends on histological findings. The highest incidence of the reported cases occurred during the sixth decade of life. The head, neck, and trunk are the most frequently reported primary locations. Ocular adnexal adenocarcinomas have a significant rate of metastasis to regional lymph nodes as well as distant sites. Standard treatment of care includes surgical excision of the tumor; however, these malignancies have a tendency to recur locally. The authors present a 14-year-old Caucasian girl with no significant past medical history who presented with a recurrent right lower eyelid tumor. She previously had multiple resections at an outside hospital; however, the margins could not be cleared. The patient was taken to the operating room for Mohs-type resection of the primary site as well as a right parotidectomy with facial nerve preservation and right modified radical neck dissection. The final pathology was consistent with a mucin-producing malignant tumor of ocular adnexa. Due to the great potential for local recurrence and metastases, the prognosis for ocular adnexal adenocarcinoma remains poor even with aggressive surgical management. The use of Mohs surgery for resection has increased as it has been linked to prolonged intervals of metastatic-free disease.     

Benign chondroid syringoma of the orbit: a rare cause of exophtalmos

Chondroid syringoma (CS) of the orbit is an extremely rare benign neoplasm. To the best of our knowledege, this is the second case reported in the english litérature.We report a case of a 41-year-old woman with orbital CS. This tumor developed slowly over 8 years causing indolor, no axil, exophtalmos of the left eye. Computed tomography demonstrated an isodense intraorbital tumor with homogeneous enhancement without bony erosion. On Magnetic resonance imaging the tumor was isointense on T1-weighted imaging, slightly hyper intense on T2-weighted imaging, and enhanced after Gadolinium administration. The patient was operated via left lateral orbitotomy. At surgery the mass was well circumscribed, extraconal, very firm and did not invade or adhere to other structures. The tumor was removed in toto. The diagnosis was confirmed by histopathological examination, the lesion was nodular, and there was differentiation toward the adnexal ductal epithelium with chondromyxoid and adipocytic differentiation in the stroma. No recurrence was seen with one year follow-up.CS should be included in the differential diagnosis of intra-orbital tumors. Complete resection remains the best therapeutic option to prevent recurrence. Close followup is recommended because malignant transformation, although rare, is possible.     

The sarcomatous guise of cervical ectopic hamartomatous thymoma

BACKGROUND: Ectopic hamartomatous thymoma is a rare benign neoplasm occurring in the deep soft tissues adjacent to the sternoclavicular joint. Although clinical presentation and diagnostic imaging can be consistent with a malignant lesion such as a sarcoma, recognition of pathologic features can readily exclude such a diagnosis. However, this remains a challenge caused by their unusual histologic features and diverse composition. Recognition of this tumor is, however, important, because it follows a benign clinical course, and conservative surgical excision is the therapy of choice. METHODS: A literature review of all reports of ectopic hamartomatous thymoma was undertaken and compared with our indexed case. RESULTS: From this review, we identified 26 previous reports. Most patients were men, with a 4.5:1 predominance. There was a wide range of ages at presentation, from 26 to 79 years. All tumors were located in the lower neck, with the exception of one arising presternally. Treatment predominantly consisted of tumor resection, with no reported recurrences on follow-up. CONCLUSIONS: We conclude that ectopic hamartomatous thymoma is an extremely rare neoplasm usually presenting in the lower neck. Correct identification of this tumor is important, because it follows a benign course, and surgical excision is adequate therapy.     

Microcystic adnexal carcinoma: an immunohistochemical study including markers of proliferation and apoptosis

Microcystic adnexal carcinoma (MAC) is the prototype for a subset of locally aggressive adnexal carcinomas (LAACs). Ultraviolet radiation (UVR) and UVB signature p53 mutations are implicated in the etiology of the most common cutaneous carcinomas. However in MACs, the role of UVR and p53 mutations is unknown. In addition, controversy still exists regarding the patterns of differentiation within these tumors. The objective of this study was to determine the expression patterns of immunohistochemical markers for p53, Ki-67, c-erbB-2, and Bcl-2 in MACs, and to compare these patterns with two MAC histologic stimulants: sclerosing type basal cell carcinomas (sBCCs) and desmoplastic trichoepitheliomas (dTEs). Other objectives were to compare expression patterns of cytokeratin (CK) AE1/AE3, CK7, CD20, endothelial membrane antigen (EMA), Ber-EP4, CD34, alpha-smooth muscle actin (SMA), and S-100 protein in MACs with its histologic simulators, and to determine the usefulness of all the immunohistochemical studies in diagnosis. Immunohistochemical markers were performed on 10 MACs, 10 sBCCs, and four dTEs. They included p53, Ki-67, c-erbB-2, Bcl-2, CK AE1/AE3, CK7, CD20, EMA, Ber-EP4, CD34, S-100 protein, and alpha-SMA. MACs expressed p53 in less than 25% of the tumor cells in only two cases (20%), and both cases showed only moderately intense staining, whereas 80% of the sBCCs were positive and showed intense staining, and all dTEs were negative. In MACs, less than 5% of the tumor cells were Ki-67 positive, whereas the sBCCs showed 20% to 40% Ki-67-positive tumor cells and dTEs showed rare Ki-67-positive cells. Bcl-2 was expressed focally in MACs, diffusely in sBCCs, and in scattered cells in dTEs. All tumors were negative for c-erbB-2. CD34, CK7, EMA, Ber-EP4, S-100 protein, and alpha-SMA all showed a distinctive pattern of staining in MACs. Although MACs arise commonly in chronically sun-exposed skin, increased expression of p53 is not found frequently. Overexpression of c-erbB-2 does not appear to be a factor in the development and progression of these adnexal tumors. Bcl-2 is expressed in MACs, but not diffusely as in sBCCs. The low level of Ki-67 supports a low proliferative rate, and other immunohistochemical markers support divergent patterns of adnexal differentiation in MACs. Immunohistochemical studies may help to differentiate MAC from sBCCs and dTEs.