Ann Otol Rhinol LaryngollOO:1991
IMAGING CASE STUDY OF THE MONTH
BONE MARROW INVASION OF MICROCYSTIC ADNEXAL CARCINOMA WILLIAM
T. C. YUH, MD, MSEE
JOHN D. ENGELKEN
DUANE C. WHITAKER, MD
KENNETH D. DOLAN, MD IOWA CITY, IOWA
of the face to irradiation. Punch biopsy and subsequent wedge excision of the lip revealed a MAC with involvement of the surgical margins. The area was treated with 57.5 Gy of radiotherapy. Three years later, lip contracture and ulceration at the surgical site led to the diagnosis of MAC recurrence.
INTRODUCTION
Microcystic adnexal carcinoma (MAC) has been shown to be locally aggressive and invade deeply and extensively without clinically apparent findings. Direct bone invasion and neural spread through the mental foramen to involve and replace the bone marrow represent a new facet in the extent of local MAC aggressiveness. We report computed tomography (CT) and magnetic resonance imaging findings of a case of MAC with bone marrow involvement due to perineural extension through the mental foramen.
A CT scan (Fig 1) of the mandible showed a broad flat plaque of enhancing tissue on the left side of the lower lip and chin that merged with the adjacent portion of the mandible and on one cut seemed to be associated with a small scalloped focal area of bony erosive change. This area was thought to represent a possible extension of the lesion into the mandibular neural canal. No spread of the tumor to lymph nodes or bone marrow was visualized.
CASE REPORT
A 51-year-old man presented with a 5-year history of an asymptomatic, firm, slightly elevated, pale yellow nodule (0.5 em) on the left side of the lower lip. There was no appreciable lymphadenopathy or other skin lesions and no history of exposure
Magnetic resonance imaging (Fig 2) revealed a defect seen in the left side of the lower lip with absent signal on intermediate and T2-weighted images. There was decreased signal in the left middle
Fig 1. Axial computed tomograms with O.8-cmslice thickness obtained through mandible from A) cranial to B) caudal show bony erosion of bony cortex (arrow, B) without apparent evidence of bone marrow involvement. From the Departments of Radiology (Yuh, Engelken, Dolan) and Dermatology (Whitaker), The University of Iowa College of Medicine, Iowa City, Iowa. REPRINTS - William T. C. Yuh, MD, MSEE, Dept of Radiology, The University of Iowa Hospitals & Clinics, Iowa City, IA 52242.
601
Downloaded from aor.sagepub.com at Bobst Library, New York University on June 29, 2015
Yuh et al, Imaging Case Study of the Month
602
Fig 2. Axial T2-weighted images (TR 2,233, TE 80) closely corresponding to Fig lA and B, respectively, show absence of normal bone marrow signal in left side of mandible (arrows) as compared with normal right side.
two thirds of the mandible that did not cross the midline and appeared to stop before the angle of the mandible. Tumor invasion of the bone marrow was demonstrated, but no lymph nodes were visibly enlarged. The patient subsequently underwent a Mohs microscopically controlled procedure to obtain tumorfree peripheral soft tissue margins. There was direct tumor invasion of the bone, grossly seen as bone surface pitting and superficial erosion. A partial hemimandibulectomy was performed, and 61.2 Gy of radiotherapy were given.
Of the 33 literature cases of MAC, 29 have appeared on the face, with the lip being the most common site. Although it was originally described as occurring primarily in women, 17 of the cases occurred in women and 16 occurred in men. Ages ranged from 18 to 76 years with a mean of 47 years. Clinically, the lesions were described as yellow, erythematous or flesh-colored, firm, indurated plaques or nodules. The size of the tumors ranged from 1 to 9 em with an average of 2 em at the time of diagnosis. The tumors had been present for 1 month to 30 years before diagnosis. 2
DISCUSSION
The locally aggressive and destructive nature of this neoplasm has been well documented. It has been shown to invade muscle, vascular adventitia, nerves, nerve sheaths, perichondrium, and periosteurn." Treatment has included local wide excision, irradiation alone, excision followed by irradiation, and the Mohs procedure. Cooper et a1 4 reported that 9 of 15 patients had at least one recurrence after the initial surgical excision.
Microcystic adnexal carcinoma is a rare, locally aggressive adnexal neoplasm initially described in 1982 by Goldstein et a1. 1 Synonyms for this neoplasm in the literature include sclerosing sweat duct carcinoma, malignant syringoma, combined adnexal tumor of the skin, and sweat duct carcinoma with syringomatous features. 2
Microcystic adnexal carcinoma has a tendency to spread along nerves, and this spread has been a prominent feature in all cases. When it invades in this manner, passage into the skull and central nervous system is possible. It also has a propensity for extensive direct local invasion without clinically apparent symptoms. These findings indicate this neo-
Direct tumor invasion of the bone and neural spread of the carcinoma along the mental nerve and inferior alveolar nerve several centimeters into the ramus of the mandible were observed microscopically. Eighteen months after surgical excision and radiotherapy, the patient shows no signs of tumor recurrence.
Downloaded from aor.sagepub.com at Bobst Library, New York University on June 29, 2015
Yuh et al, Imaging Case Study oj the Month
plasm is much more aggressive than most cutaneous neoplasms. The neoplasm invaded deep tissue layers in every case, and muscle was involved in many of the cases." Direct bone invasion, bone marrow replacement by tumor, and metastases have not been described previously. Bone has been resistant to most types of skin cancers. However, when bone invasion does occur, it is usually via a foramen or osseous suture line, or in an area of focal bone injury." The high incidence of recurrence may be secondary to the failure to recognize the tendency for aggressive and extensive invasion by these tumors without clinical radiographic evidence of tumor in-
603
volvement. Thus, the surgical excision and/or irradiation treatment given to these patients may be inadequate. Tumor spread well beyond the clinically apparent lesion and invasion into the skull are thus important issues to assess. In our case, focal bone erosion secondary to direct tumor invasion was detected by CT scan; however, CT failed to detect the extensive bone marrow replacement by tumor. Magnetic resonance imaging has been proven to be valuable in the detection of bone marrow disease. Although bony structure and perineural involvement was not obvious in our case, magnetic resonance imaging clearly demonstrated the abnormal bone marrow signal due to tumor infiltration preoperatively.
REFERENCES 1. Goldstein OJ, Barr RF, Santa Cruz DJ. Microcystic adnexal carcinoma: a distinct clinicopathologic entity. Cancer 1982; 50:566-72. 2. Chow WC, Cockerell CJ, Geronemus RG. Microcystic adnexal carcinoma of the scalp. J Oermatol Surg Oncol 1989;15: 768-71. 3. Cooper PH, Mills SEe Microcystic adnexal carcinoma.
J
Am Acad Dermatol 1984;10:908-14. 4. Cooper PH, Mills SE, Leonard 00, et aI. Sclerosingsweat duct (syringomatous) carcinoma. Am J Surg Pathol 1985;9:42233. 5. Mohs FE. Chemosurgery: microscopically controlled surgery for skin cancer. Springfield, Ill: Charles C Thomas, 1978.
TWENTY-SEVENTH POSTGRADUATE COURSE IN EAR SURGERY The 27th Postgraduate Course in Ear Surgery will be held from April 26 to May 1, 1992, in Nijmegen, the Netherlands. For further information and registration form, please write to Prof Or P. van den Brock, University Hospital Nijmegen, Department of Otorhinolaryngology, PO Box 9101, 6500 HB Nijmegen, the Netherlands; Fax: 80-540251.
Downloaded from aor.sagepub.com at Bobst Library, New York University on June 29, 2015
IMAGING CASE STUDY OF THE MONTH
BONE MARROW INVASION OF MICROCYSTIC ADNEXAL CARCINOMA WILLIAM
T. C. YUH, MD, MSEE
JOHN D. ENGELKEN
DUANE C. WHITAKER, MD
KENNETH D. DOLAN, MD IOWA CITY, IOWA
of the face to irradiation. Punch biopsy and subsequent wedge excision of the lip revealed a MAC with involvement of the surgical margins. The area was treated with 57.5 Gy of radiotherapy. Three years later, lip contracture and ulceration at the surgical site led to the diagnosis of MAC recurrence.
INTRODUCTION
Microcystic adnexal carcinoma (MAC) has been shown to be locally aggressive and invade deeply and extensively without clinically apparent findings. Direct bone invasion and neural spread through the mental foramen to involve and replace the bone marrow represent a new facet in the extent of local MAC aggressiveness. We report computed tomography (CT) and magnetic resonance imaging findings of a case of MAC with bone marrow involvement due to perineural extension through the mental foramen.
A CT scan (Fig 1) of the mandible showed a broad flat plaque of enhancing tissue on the left side of the lower lip and chin that merged with the adjacent portion of the mandible and on one cut seemed to be associated with a small scalloped focal area of bony erosive change. This area was thought to represent a possible extension of the lesion into the mandibular neural canal. No spread of the tumor to lymph nodes or bone marrow was visualized.
CASE REPORT
A 51-year-old man presented with a 5-year history of an asymptomatic, firm, slightly elevated, pale yellow nodule (0.5 em) on the left side of the lower lip. There was no appreciable lymphadenopathy or other skin lesions and no history of exposure
Magnetic resonance imaging (Fig 2) revealed a defect seen in the left side of the lower lip with absent signal on intermediate and T2-weighted images. There was decreased signal in the left middle
Fig 1. Axial computed tomograms with O.8-cmslice thickness obtained through mandible from A) cranial to B) caudal show bony erosion of bony cortex (arrow, B) without apparent evidence of bone marrow involvement. From the Departments of Radiology (Yuh, Engelken, Dolan) and Dermatology (Whitaker), The University of Iowa College of Medicine, Iowa City, Iowa. REPRINTS - William T. C. Yuh, MD, MSEE, Dept of Radiology, The University of Iowa Hospitals & Clinics, Iowa City, IA 52242.
601
Downloaded from aor.sagepub.com at Bobst Library, New York University on June 29, 2015
Yuh et al, Imaging Case Study of the Month
602
Fig 2. Axial T2-weighted images (TR 2,233, TE 80) closely corresponding to Fig lA and B, respectively, show absence of normal bone marrow signal in left side of mandible (arrows) as compared with normal right side.
two thirds of the mandible that did not cross the midline and appeared to stop before the angle of the mandible. Tumor invasion of the bone marrow was demonstrated, but no lymph nodes were visibly enlarged. The patient subsequently underwent a Mohs microscopically controlled procedure to obtain tumorfree peripheral soft tissue margins. There was direct tumor invasion of the bone, grossly seen as bone surface pitting and superficial erosion. A partial hemimandibulectomy was performed, and 61.2 Gy of radiotherapy were given.
Of the 33 literature cases of MAC, 29 have appeared on the face, with the lip being the most common site. Although it was originally described as occurring primarily in women, 17 of the cases occurred in women and 16 occurred in men. Ages ranged from 18 to 76 years with a mean of 47 years. Clinically, the lesions were described as yellow, erythematous or flesh-colored, firm, indurated plaques or nodules. The size of the tumors ranged from 1 to 9 em with an average of 2 em at the time of diagnosis. The tumors had been present for 1 month to 30 years before diagnosis. 2
DISCUSSION
The locally aggressive and destructive nature of this neoplasm has been well documented. It has been shown to invade muscle, vascular adventitia, nerves, nerve sheaths, perichondrium, and periosteurn." Treatment has included local wide excision, irradiation alone, excision followed by irradiation, and the Mohs procedure. Cooper et a1 4 reported that 9 of 15 patients had at least one recurrence after the initial surgical excision.
Microcystic adnexal carcinoma is a rare, locally aggressive adnexal neoplasm initially described in 1982 by Goldstein et a1. 1 Synonyms for this neoplasm in the literature include sclerosing sweat duct carcinoma, malignant syringoma, combined adnexal tumor of the skin, and sweat duct carcinoma with syringomatous features. 2
Microcystic adnexal carcinoma has a tendency to spread along nerves, and this spread has been a prominent feature in all cases. When it invades in this manner, passage into the skull and central nervous system is possible. It also has a propensity for extensive direct local invasion without clinically apparent symptoms. These findings indicate this neo-
Direct tumor invasion of the bone and neural spread of the carcinoma along the mental nerve and inferior alveolar nerve several centimeters into the ramus of the mandible were observed microscopically. Eighteen months after surgical excision and radiotherapy, the patient shows no signs of tumor recurrence.
Downloaded from aor.sagepub.com at Bobst Library, New York University on June 29, 2015
Yuh et al, Imaging Case Study oj the Month
plasm is much more aggressive than most cutaneous neoplasms. The neoplasm invaded deep tissue layers in every case, and muscle was involved in many of the cases." Direct bone invasion, bone marrow replacement by tumor, and metastases have not been described previously. Bone has been resistant to most types of skin cancers. However, when bone invasion does occur, it is usually via a foramen or osseous suture line, or in an area of focal bone injury." The high incidence of recurrence may be secondary to the failure to recognize the tendency for aggressive and extensive invasion by these tumors without clinical radiographic evidence of tumor in-
603
volvement. Thus, the surgical excision and/or irradiation treatment given to these patients may be inadequate. Tumor spread well beyond the clinically apparent lesion and invasion into the skull are thus important issues to assess. In our case, focal bone erosion secondary to direct tumor invasion was detected by CT scan; however, CT failed to detect the extensive bone marrow replacement by tumor. Magnetic resonance imaging has been proven to be valuable in the detection of bone marrow disease. Although bony structure and perineural involvement was not obvious in our case, magnetic resonance imaging clearly demonstrated the abnormal bone marrow signal due to tumor infiltration preoperatively.
REFERENCES 1. Goldstein OJ, Barr RF, Santa Cruz DJ. Microcystic adnexal carcinoma: a distinct clinicopathologic entity. Cancer 1982; 50:566-72. 2. Chow WC, Cockerell CJ, Geronemus RG. Microcystic adnexal carcinoma of the scalp. J Oermatol Surg Oncol 1989;15: 768-71. 3. Cooper PH, Mills SEe Microcystic adnexal carcinoma.
J
Am Acad Dermatol 1984;10:908-14. 4. Cooper PH, Mills SE, Leonard 00, et aI. Sclerosingsweat duct (syringomatous) carcinoma. Am J Surg Pathol 1985;9:42233. 5. Mohs FE. Chemosurgery: microscopically controlled surgery for skin cancer. Springfield, Ill: Charles C Thomas, 1978.
TWENTY-SEVENTH POSTGRADUATE COURSE IN EAR SURGERY The 27th Postgraduate Course in Ear Surgery will be held from April 26 to May 1, 1992, in Nijmegen, the Netherlands. For further information and registration form, please write to Prof Or P. van den Brock, University Hospital Nijmegen, Department of Otorhinolaryngology, PO Box 9101, 6500 HB Nijmegen, the Netherlands; Fax: 80-540251.
Downloaded from aor.sagepub.com at Bobst Library, New York University on June 29, 2015
