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Ryan Fitzgerald, DPM
PRESENT RI Associate Editor
Hess Orthopedics &
Sports Medicine
Harrisonburg, Virginia
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Case Conclusion:
Plantar Soft Tissue Mass
in the Pediatric Patient
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Click the image for larger view |
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In last week's Residency Insight, we reviewed the clinical exam and the physical and imaging findings, to determine how best to proceed with the case. There were a couple of strong responses in the eTalk — so please feel free to chime in at the conclusion of tonight's eZine. You're participation is always welcome.
Conclusion
Following removal of the soft tissue mass, the patient presented in one week for follow up. He stated that he continued to comply with the daily dressing changes and non-weight bearing status. His wound was clean and well coapted with the sutures intact; there was no maceration or drainage noted. The patient related to having no further pain or discomfort. Review of the pathology report demonstrated that the 1 cm2 mass was a benign epidermal cyst. The patient was instructed to follow-up in one week (total two weeks post-op) at which time the sutures were removed, and the patient was transitioned into tennis shoes with gradual increases in weight-bearing activity. To date, the patient is approximately six weeks following removal of the soft tissue mass in his right foot; he is experiencing no pain, and has returned to full activity and participation in sports.
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The Banquet Film - WesternU DPM 2013 (HD)
Want to remember back to those times in Podiatric Medical School? Remember the stress, the tests, the friends? Here's a video produced by the current third year class of Western University's College of Podiatric Medicine as a retrospective view of their time as podiatrists to be. Follow this link, or scroll down at the conclusion of this eZine, for an intro from the filmmaker, Michael Corpuz, and a link to view the video. |
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Discussion
In this particular case presentation, the presumptive diagnosis of epidermal cyst was obtained via the physical exam and confirmation of the diagnosis was made through pathological evaluation of the specimen.
Epidermal cysts, often also termed epidermal inclusion cysts or epidermoid cysts, are benign, slow growing cysts which contain keratinous, follicular, or sebaceous material. Often, this materially has a fetid and ‘cheesy’ quality which can be helpful in making the diagnosis. These benign lesions are most often found on the scalp, ears, face, back and scrotum, however presentation in the lower extremity can occur. Generally speaking, epidermal cysts are twice as more likely to occur in men than women, and of the presenting age is most commonly in the 3rd or 4th decade of life, although research has demonstrated that epidermal cysts can occur at any age. Gardner’s syndrome is the exception, and in these patients’, epidermal cysts generally begin to form around the age of puberty, with the average age being 13 years.
Epidermal cysts result from the proliferation of epidermal cells within a portion of the dermis. Of then these epidermal cells are directly inoculated into the dermis via trauma, however there is some evidence to suggest that there is a hereditary component in certain patients, especially in those patients with a positive family history. In these instances, cyst formation is likely do to derangement of germ layer formation during development.
Upon physical exam, epidermal cysts appear as flesh colored, superficial, firm, globular papules or nodules that are often freely movable against the deeper subcutaneous tissue. In many cases, a small pore or sinus tract is appreciated. Often these soft tissue masses are nontender, although they can become painful if located in a weight-bearing area or if they become infected. Histologically, these cysts appear as stratified squamous epithelium that contains a granular layer and is filled with keratinous material that is often in a laminated arrangement (fig.1).
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Fig. 1: Granular epithelial cells line the epidermal cyst while lamellar keratinous material is noted within the central portion of the cyst. |
Asymptomatic epidermal cysts require no treatment, however should these lesions become painful, they can be injected with steroid, evacuated via incision and drainage, or removed surgically.
Gardner’s Syndrome
In patients who present with lower extremity epidermal cysts, it is important that one evaluate the patient for Gardner syndrome, especially when treating a pediatric patient. Gardner’s syndrome, which is a variant of familial adenomatous polyposis (FAP), is an autosomal dominant disease that occurs about 1 in every 8,000 people, and is characterized by gastrointestinal polyps, multiple osteoma formation, and skin and soft tissue tumors. Often, cutaneous findings, such as epidermal cysts, are the initial presenting symptoms. In these patients, epidermal cyst formation occurs earlier, around the age of puberty, and can be seen as a sentinel event in the natural history of FAP. Early diagnosis is critical; studies have demonstrated that polyps in Gardner’s syndrome have 100% of undergoing malignant transformation (fig. 2).
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Fig. 2: On CT, the polyp becomes visible in a patient with Gardner’s syndrome. |
Upon physical exam, many skin findings can be observed; additionally it is important to note the age of onset of lesions, and to establish if other members of the family have similar lesions. To further evaluate the patient for the possibility of Gardner’s syndrome, it is necessary to perform thyroid screening via physical exam and ultrasonography to evaluate for the presence of thyroid tumors. Radiographs should be obtained from the mandible and the long bones to evaluation for the presence of osteomas and hyperostosis.
Additionally, ophthalmologic examination can be useful to detect pigmented lesions of the fundus, which, in addition to skin lesions, are often the initial presenting sign of disease.
In patients where the clinical suspicion is high, it is necessary to perform fecal occult blood tests and sigmoidoscopy and colonoscopy to evaluate for the presence of polyp formation. It is recommended that the patient undergo these tests every 1-2 years until the age of fifty to monitor the disease. In patients with Gardner’s syndrome, due to the high likelihood of malignant transformation of the colon polyps, resection of portions of the colon is often indicated, this is especially so if thirty or more polyps are identified during colonoscopy.
Final Thought
In the case presented, of particular concern was the age of the patient. While epidermal cysts can occur at any age, generally speaking a younger presenting age significantly increases the clinical suspicion for Gardner’s Syndrome. Despite having a negative family history, the patient was referred back to his primary care doctor to be thoroughly evaluated for Gardner’s syndrome. It is vitally important that we, as foot and ankle surgeons recognize that many life-threatening conditions can have their initial symptoms in the lower extremity. As lower extremity specialists, we must be prepared adequately recognize these manifestations, and treat these patients or make referrals appropriately. In doing so, we will save lives.
Follow this link to the eTalk topic on this case presentation and share any final thoughts you may have.
With your continued participation, we can greatly increase our collective knowledge and therefore make us all better physicians and surgeons.
Keep writing in with your thoughts and comments. Better yet, post them in our eTalk forum.
Sources:
Ascari-Raccagni A, Baldari U, Righini MG. Cutaneous symptoms of Gardner''s syndrome. J Eur Acad Dermatol Venereol. Jan 1999;12(1):80-1.
Cotran RS, Kumar V, Collins T. Pathologic Basis of Disease. Philadelphia, Pa: WB Saunders; 1999:. 831.
Lee HE et al. Comparison of the surgical outcomes of punch incision and elliptical excision in treating epidermal inclusion cysts: a prospective, randomized study. Dermatol Surg. 2006 Apr;32(4):520-5.
Narisawa Y, Kohda H. Cutaneous cysts of Gardner''s syndrome are similar to follicular stemcells. J Cutan Pathol. Apr 1995;22(2):115-21
Sukal SA et al. Safer and less unpleasant incision and drainage of epidermal inclusion cysts. Dermatol Surg. 2006 Sep;32 (9):1214-5
Van Epps KJ, Kuszyk BS, Hofmann LV, Fishman EK. Epidermoid inclusion cysts seen on CT of a patient with Gardner''s syndrome. AJR Am J Roentgenol. Sep 1999;173(3):858-9.
The Banquet Film - WesternU DPM 2013 (HD)
At the College of Podiatric Medicine at WesternU, It has become a tradition for the charter class to prepare a quick video for the student and faculty end-of-the-year banquet. This past May, with the stresses of the academic year behind them and the excitement of the next phase of their educational journeys building, the Class of 2013 relished in another opportunity to flex their creative muscle and display their comical camaraderie.
This short film lightheartedly recaps the gags and inside jokes that the class shares with one another, and answers some burning questions such as: how do you inspire and motivate an already mentally-taxed student? What makes a good (and bad) doctor in the clinical setting? And what if residencies selected students via a draft?
Watch it to witness the well-roundedness, wit, and humor of WesternU's charter class. Watch it for its praiseworthy thespian performances. Watch it in horror as you realize that these individuals are the doctors of tomorrow. But most of all, just watch it for the laughs.
Thank you, and enjoy!
—Michael Corpuz
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