A Case of Primary Mucinous Carcinoma of the Scalp Treated With Mohs Surgery

BACKGROUND: Primary mucinous carcinoma of the skin is a rare sweat gland malignancy that is associated with locally aggressive behavior and a high rate of local recurrence following simple excision. OBJECTIVE: A patient with primary mucinous carcinoma of the scalp, which was treated with Mohs micrographic surgery (MMS), is described. METHODS: Case report and literature review. RESULTS: The patient underwent MMS to remove the tumor. Thirty months after the procedure, the patient remains tumor free. CONCLUSION: Simple excision of primary mucinous carcinoma of the skin is associated with a high recurrence rate. Given the low rate of metastasis and characteristic histologic tumor continuity associated with primary mucinous carcinoma of the skin, as well as the tendency for the tumor to involve cosmetically sensitive areas, such as the face and eyelids, MMS appears to represent a preferable treatment alternative for this particular sweat gland tumor. MMS appears to be associated with a very low risk of tumor recurrence.     

Mohs'' Micrographic Surgery of a Proliferating Trichilemmal Tumor in a Young Black Man

BACKGROUND: Proliferating trichilemmal tumor is an uncommon tumor of the follicular isthmus of the hair follicle. It usually presents as a solitary nodule on the scalp of older white women. Although these lesions typically behave in a benign fashion, recurrences and metastasis after local excision have been reported. Mohs' micrographic surgery has been effectively used to treat adnexal neoplasms. OBJECTIVE: To report a case of a proliferating trichilemmal tumor in a young black man, which was excised using Mohs' micrographic surgery. METHODS: Case report and review of the literature. RESULTS: Mohs' micrographic surgery demonstrated an irregular extension of the tumor beyond a 1 cm surgical margin. CONCLUSIONS: Proliferating trichilemmal tumors should be considered in the differential diagnosis of cutaneous neoplasms on the scalp in persons of any age (with the possible exception of infants and children), sex, or race. Mohs' micrographic surgery may be considered an optimal treatment option for proliferating trichilemmal tumors because these lesions may have an infiltrative component that may not be clinically apparent.     

Mohs Micrographic Surgery of Primary Cutaneous Mucinous Carcinoma Using Immunohistochemistry for Margin Control

BACKGROUND: Primary cutaneous mucinous carcinoma is a rare adnexal malignancy with a high recurrence rate following conventional excision and the potential for aggressive local invasion. OBJECTIVE: To enhance the microscopic detection of mucinous carcinoma in Mohs micrographic surgical sections by incorporating rapid immunohistochemical staining. METHODS: Standard Mohs micrographic surgical technique was used in conjunction with frozen section immunohistochemistry using an antibody to low-molecular-weight cytokeratin. RESULTS: Rapid immunoperoxidase staining using low-molecular-weight cytokeratin detected residual foci of mucinous carcinoma that were difficult to identify on routine frozen sections. Immunostaining was strongly positive in areas with clear evidence of tumor by routine histology, as well as in adjacent areas on a subsequent stage where frozen sections were equivocal or negative. Immunostaining was distinctly negative at the final surgical margin, which was shown by en face permanent sections to be free of tumor. The patient has been free of recurrence for 3 years. CONCLUSION: Immunoperoxidase-guided Mohs micrographic surgery using low-molecular-weight cytokeratin enhances the sensitivity for detection of mucinous carcinoma, and may help contribute to complete tumor removal.     

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.     

Curettage prior to Mohs'' Micrographic Surgery for Previously Biopsied Nonmelanoma Skin Cancers: What Are We Curetting? Retrospective, Prospective, and Comparative Study

BACKGROUND: Curettage prior to excision and Mohs' micrographic surgery for nonmelanoma skin cancer is performed based on the assumption that the curette will remove softer, more friable tumor-infiltrated dermis and leave structurally intact normal skin. This assumption, however, has not been objectively examined in the dermatologic surgery literature. OBJECTIVE: We performed a study to examine the ability of curettage to selectively remove and delineate nonmelanoma skin cancer prior to Mohs' micrographic surgery. METHODS: The study included 150 previously biopsied basal cell and squamous cell carcinomas less than 1.5 cm in size. We conducted (1) a retrospective study of 50 tumors curetted prior to Mohs' surgery by a surgeon who routinely curettes preoperatively; (2) a prospective study in which a surgeon who routinely does not curette preoperatively curetted 50 tumors prior to Mohs' surgery; and (3) a comparative historical group of 50 noncuretted tumors treated with Mohs' surgery by the latter surgeon. All curetted tissue was evaluated histologically. RESULTS: Only 50% of the curetted tissue demonstrated the presence of tumor in the curettings, but in 76% of these, the curette left residual tumor at the surgical margins. Of the other 50% in which the curette removed only non-cancer-containing skin, 34% had tumor present at the surgical margin. Overall, the curette removed tumor, leaving no residual tumor at the surgical margins in only 12% of lesions. Comparison with historical noncuretted tumors operated on by the same surgeon showed that curettage did not affect the mean number of stages or the proportion of tumors requiring more than one stage for histologic clearance. CONCLUSION: Although curettage may be helpful in debulking friable skin prior to Mohs' micrographic surgery, it does not reliably delineate the extent of a tumor.     

Sebaceous Carcinoma of the Eyelid: A Review of 14 Cases

BACKGROUND: Sebaceous carcinoma of the eyelid is a rare tumor. Treatment can be complicated by noncontiguous spread of the tumor. OBJECTIVE: Review a series of patients with sebaceous carcinoma to illustrate clinical presentations, treatments, and outcomes. METHODS: We retrospectively reviewed medical records of patients with sebaceous carcinoma treated at Mayo Clinic (Rochester, MN). RESULTS: Fourteen patients had sufficient follow-up data available for review. Mean follow-up was 57 months (range 18-134 months). Treatment included wide local excision with frozen and permanent section control (9 patients, 64%), Mohs micrographic surgery (2 patients, 14%), external beam radiation (2 patients, 14%), and exenteration and total parotidectomy with cervical lymph node dissection (1 patient, 7%). Two patients (14%) had local recurrence of the tumor after wide local excision, and 1 patient (7%) had tumor recurrence after Mohs micrographic surgery. CONCLUSION: Treatment should be chosen on the basis of the extent of the tumor and the specific needs of the patient. The mainstay of treatment of tumors without orbital involvement has been wide local excision, with the margins checked in both permanent and frozen sections, in combination with conjunctival map biopsies when warranted. Mohs micrographic surgery is an alternative that may provide tissue conservation and lower recurrence rates. Recurrence rates between treatments are difficult to assess because of the small number of cases reported in the literature. In cases with orbital involvement, exenteration may be warranted. Radiation may be useful when surgery cannot be tolerated.     

Peripheral in-continuity tissue examination: a modification of Mohs' micrographic surgery

The use of peripheral in-continuity tissue examination, in which the surgeon or dermatologist and pathologist combine their talents to remove cutaneous tumors with rapid evaluation of all margins, is useful and practical for excision of basal cell and squamous cell carcinomas that are recurrent, large, or occur in historically difficult areas to treat. This procedure, like Mohs' micrographic surgery, can be used as a tissue-sparing measure while still providing the physician and patient with confidence of complete tumor removal. Because the technique allows for primary closure and immediate reconstruction of larger and more complex tumors, patients can be spared the added inconvenience, pain, and expense of multiple separate procedures that may be necessary with Mohs' micrographic surgery or traditional tumor excision with permanent section margin evaluation.     

Trichilemmal Carcinoma: A Rare Cutaneous Malignancy: A Report of Two Cases

BACKGROUND: Trichilemmal carcinoma is a rare cutaneous malignancy that usually occurs on the sun-exposed areas of older individuals. The lesion is usually solitary and may present as an exophytic or polypoid nodule that maybe hyperkeratotic with ulceration. OBJECTIVE: To present two cases of trichilemmal carcinoma, one occurring in a kidney transplantation patient. METHODS: Two case reports and a discussion of the rare carcinoma are presented. RESULTS: Both lesions were treated with Mohs micrographic surgery without sign of recurrence after several years. CONCLUSION: Trichilemmal carcinoma is a rare cutaneous malignancy that can be seen in both immunocompetent and immunosuppressed hosts. Mohs micrographic surgery should be considered among the surgical options to avoid a wide surgical excision in these patients.     

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.     

5% Imiquimod Cream and Reflectance-Mode Confocal Microscopy as Adjunct Modalities to Mohs Micrographic Surgery for Treatment of Basal Cell Carcinoma

Background. Imiquimod is an immune response modifier that up-regulates cytokines and has been shown in clinical studies to reduce or clear basal cell carcinoma tumors when applied topically.
Objective. The objectives were to evaluate the efficacy of 5% imiquimod cream in treating basal cell carcinoma preceding excision by Mohs micrographic surgery and to determine if reflectance-mode confocal microscopy is useful to establish the need for surgical intervention after imiquimod treatment.
Methods. Subjects applied study cream to one biopsy-confirmed basal cell carcinoma tumor 5 ×/week for 2, 4, or 6 weeks in this vehicle-controlled, double-blind study. Confocal microscopy was used for the 6-week treatment group to examine the target tumor area at each interval visit and immediately before Mohs micrographic surgery. After the Mohs micrographic surgery excision, the tissue was evaluated histologically, and the excision area was measured. Confocal microscopy readings were correlated to the histologic diagnosis.
Results. Tumors cleared or the target tumor area was reduced in subjects in the 4- and 6-week dosing regimens. Confocal microscopy assessments correlated well with the histologic diagnosis.
conclusion. Imiquimod improved excision results relative to vehicle when used for treating basal cell carcinoma before Mohs micrographic surgery. Confocal microscopy assessments correlated well with tumor response to therapy, suggesting that confocal microscopy may help determine the need for surgery.     

Bilateral Primary Mucinous Carcinoma of the Eyelid Treated with Mohs Surgery

BACKGROUND: Primary mucinous carcinoma is an uncommon malignant cutaneous tumor which arises most commonly on the eyelid. While rarely causing death, recurrence following primary excision is common and widespread metastasis may occur. OBJECTIVE: We report the first case of bilateral primary mucinous carcinoma of the eyelid. METHODS: A lesion of the left lower eyelid had been resected three times previously with positive conventional margins. Both this lesion and a second primary lesion of the contralateral lower lid were removed with Mohs microscopically controlled excision without recurrence for more than 2 years. CONCLUSION: Multiple lesions of mucinous carcinoma of the eyelid do not necessarily connote metastasis. Mohs microscopically controlled excision may be a suitable form of therapy for primary mucinous carcinoma of the eyelid.     

Mohs Micrographic Surgery of a Papillary Eccrine Adenoma

BACKGROUND: Papillary eccrine adenoma (PEA) is a rare benign sweat gland neoplasm first described by Rulon and Helwig in 1977. Although these lesions typically behave in a benign fashion, PEA's on the volar surfaces may demonstrate more aggressive biologic behavior. Additionally, aggressive digital papillary adenomas (ADPA) may histologically simulate PEAs and behave in a more malignant fashion. OBJECTIVE: To present a case report of a patient with an incompletely excised PEA that was successfully extirpated using Mohs micrographic surgery (MMS). METHODS: A 51-year-old black woman was evaluated for the treatment of an incompletely excised PEA located on the dorsum of her left hand at the base of the thumb. Mohs micrographic surgery was felt to be the ideal treatment choice because of incomplete prior resections, ill-defined clinical borders, the need for conservative surgical excision to preserve sensory and motor function of the left hand, and the previously reported more aggressive nature of this tumor when located on volar surfaces. The patient underwent a two-stage, six section micrographically controlled excision using the fresh tissue technique. RESULTS: Complete resection of the PEA without significant damage to neurovascular structures. CONCLUSION: This case demonstrates the increasingly important role MMS is playing in the surgical management of a wide variety of cutaneous tumors. To our knowledge, this is the first time MMS has been used in the resection of a PEA.     

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.     

Mohs Micrographic Surgery for Squamoid Eccrine Ductal Carcinoma

Background. Squamoid eccrine ductal carcinoma is a rare cutaneous malignancy. There are no accepted standards for surgical margins in eccrine carcinomas.
Objective. We report a case of squamoid eccrine ductal carcinoma resembling squamous cell carcinoma and discuss Mohs micrographic surgery as a surgical modality for eccrine carcinomas.
Method. The patient was a 30-year-old Korean woman with a 4-year history of a nodule on her neck. Following primary diagnosis of squamous cell carcinoma by punch biopsy, the tumor was completely removed by Mohs micrographic surgery with a 2 mm cancer-free margin.
Result. A one-stage Mohs micrographic surgical procedure was performed, and the size of the tumor mass was 2.3 × 2.5 cm in width and 1.5 cm in depth. On histopathologic examination, the tumor was characterized by both eccrine and squamous differentiation. The squamous cells expressed epithelial membrane antigen and cytokeratin 5 and 6, and the cells forming ductal structures expressed anti–carcinoembryonic antigen. Although eccrine carcinomas show a generally aggressive clinical course, the patient was disease free at 14 months after surgery.
Discussion. Squamoid eccrine ductal carcinoma should be considered in the differential diagnosis of squamous cell carcinoma and other cutaneous adnexal neoplasms showing squamoid and ductal features of differentiation. In addition, Mohs micrographic surgery can be an option sufficient for complete surgical removal of eccrine carcinomas such as squamoid eccrine ductal carcinoma.
YONG JU KIM, MD, AE REE KIM, MD, PHD, AND DONG SOO YU, MD, HAVE INDICATED NO SIGNIFICANT INTEREST WITH COMMERCIAL SUPPORTERS.     

Syringocystadenocarcinoma Papilliferum: Successfully Treated With Mohs Micrographic Surgery

BACKGROUND: Syringocystadenocarcinoma papilliferum, a rare sweat gland carcinoma, is the malignant counterpart of syringocystadenoma papilliferum. OBJECTIVE: To demonstrate a rare case of syringocystadenocarcinoma papilliferum successfully treated with Mohs micrographic surgery. METHODS: A 60-year-old male presented with two verrucous plaques on his right auricle since childhood. These two plaques became ulcerated, more painful, and pruritic in 1 year. Histopathologic examination revealed syringocystadenocarcinoma papilliferum. RESULTS: Mohs micrographic surgery with reconstruction of right auricle was performed subsequently. There are no signs of recurrence or metastasis 6 years after operation. CONCLUSION: Syringocystadenocarcinoma papilliferum can be successfully treated with Mohs micrographic surgery.     

Mucoepidermoid/Adenosquamous Carcinoma of the Skin: Presentation of Two Cases

BACKGROUND: Mucoepidermoid carcinoma is a relatively common neoplasm of the major and minor salivary glands comprising 10-30% of primary carcinomas. They may involve the skin through direct extension, metastases, and rarely, as a primary focus (adenosquamous carcinoma). OBJECTIVE: To discuss through case reports, the nomenclature, histology, clinical course, and treatment of mucoepidermoid/adenosquamous carcinoma. METHODS: We present a case of mucoepidermoid carcinoma primary to an upper eyelid accessory lacrimal gland with direct cutaneous extension and a case of primary cutaneous adenosquamous carcinoma of the scalp. RESULTS: An eyelid neoplasm of lacrimal origin was initially treated with Mohs micrographic surgery (MMS), requiring an orbital exenteration to achieve a tumor-free plane. In the second case, a primary scalp lesion was cleared with MMS. Neither patient has had local recurrence or metastases. CONCLUSION: Correct diagnosis is crucial to pursuing adequate treatment for this aggressive neoplasm. We support the use of MMS to achieve local control.     

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.     

Invasive primary mucinous carcinoma of the skin.

An unusual case of primary mucinous carcinoma of the skin has been reported. The tumor progressed very rapidly and recurred twice within a short period after adequate local excision. It had wide spread lymph node metastases and a fatal ending. The characteristic clinical and histologic differentiation from sweat gland carcinoma has been discussed.     

Pilomatrix Carcinoma of the Back Treated by Mohs Micrographic Surgery

BACKGROUND: Pilomatrix carcinoma (synonyms, matrical carcinoma or malignant pilomatrixoma) is a rare malignant neoplasm derived from the hair matrix first described in 1980. This neoplasm can exhibit local aggressive behavior and distant metastasis. Most pilomatrix carcinomas occur on the head and neck of elderly individuals with a predilection for males (M:F 5:1). Pilomatrix carcinoma is often clinically misdiagnosed as a sebaceous cyst and histologic difficulty can occur in differentiating this entity from the benign entity pilomatrixoma. OBJECTIVE: The objective was to describe a case of pilomatrix carcinoma encountered in a Mohs micrographic surgery practice. We present the first case of this lesion treated by Mohs surgery. METHODS: A case report and literature review are presented. CONCLUSION: Pilomatrix carcinoma is a rare malignant variant of pilomatrixoma. Given the rarity of this lesion there are no well-defined standards for surgical management. Wide local excision has been recommended given the high rate of reoccurrence. Mohs micrographic surgery may provide optimal treatment of this neoplasm given the ability to have 100% margin control.     

Mucinous carcinoma of the external auditory canal: case report

An unusual case of a mucinous carcinoma originating from the external auditory canal in a 70-year-old man is described. Multiple metastatic lesions also appeared in the parotid gland. After a local excision of the primary lesion, the tumor was seen to extend anterosuperiorly along the middle cranial cavity dura. The occurrence of this tumor is exceedingly rare, and, to our knowledge, this is the first report of a mucinous carcinoma of the external auditory canal. The clinical pictures of this tumor and the histological findings are discussed with reference to other reports.