Desmoplastic Melanoma

Abstract: 83 yo man with a mixed desmoplastic melanoma of the scalp

HPI: In August 2013, this 83 yo man was seen with an 8 mm diameter nodule on the left parietal scalp present for  4 – 5 months and growing rapidly.  He had a history of two thin melanomas excised from the left forhead and left temple from 2009 – 2011.  In reviewing the biopsy reports, it can not be determined if they were both the same lesion or two separate tumors.

O/E: 8 mm pink to red well-circumscribed nodule left parietal. The dermatoscopic image shows multiple polymorphous blood vessels with central crystalline structures and a pink hue (vascular blush ) in the background.  No palpably enlarged lymph nodes.

Clinical Photos:

Dermoscopic Image - Courtesy of Yoon Cohen

Pathology:  Desmoplastic melanoma > 7.35 mm thick.  Level 5.  The tumor was/was not purely desmoplastic but over 10% of the cells has a spindle and epitheloid component as well.

Lab and Xray:

All blood studies including LDH within normal range.

P.E.T. Scan no abnormal foindings

Surgical Treatment: Patient underwent a WLE with 2 cm margins on October 2, 2013.  The defect was closed with a fasciocutaneous flap. Sentinel lymphnode biopsy was not performed.

Diagnosis: Desmoplastic melanoma with mixed histology, greater than 7.25 mm thick  No evidence of distant spread.

The patient has been seen at two centers for discussion of further treatment.  Local radiotherapy was recommended at both, although two different protocols were discussed.  The patient is undecided if he wants radiotherapy at this time.

Discussion:  The literature indicates that local radiotherapy reduces the incidence of local recurrence, but it is unclear if it provides an overall survival benefit.  At least one study suggests that patients undergoing local radiotherapy have a lower survival (ref 1 below).  It does not mention if these patients had more advanced disease that those who did not receive radiotherapy.

Questions:  What would your recommendations to this patient be?  Would you favor local radiotherapy?  See Discussion above.

References:

1. Desmoplastic melanoma - the step-child in the melanoma family?

Wasif N, Gray RJ, Pockaj BA.
J Surg Oncol. 2011 Feb;103(2):158-62.

Abstract

BACKGROUND AND OBJECTIVES: Desmoplastic melanoma (DM) is a rare variant of cutaneous melanoma. Our goal was to study the surgical management of DM, identify prognostic factors, and impact of treatment options.

METHODS: Patients with DM (n = 1,735) were identified from the Surveillance, Epidemiology, and End Results database (1988-2006).

RESULTS: The median age of the study population was 69 years and overall survival (OS) at 5 years 65%. DM was more common in males (65%), most commonly found on the head and neck (51%), and had a mean thickness of 2.97 mm. Patients undergoing a wide local excision (WLE; ≥1 cm) had improved 5-year OS compared to a simple excision (<1 cm) or biopsy alone (67% vs. 60% vs. 45%, respectively, P < 0.001). Of 505 patients (29%) undergoing sentinel node biopsy (SLNB), only 14 (2.8%) were positive. Traditional prognostic factors such as Breslow thickness, nodal positivity, and ulceration did not predict survival. On multivariate analysis only adjuvant radiation therapy [HR 1.65 (95% CI 1.17-2.31)] and WLE correlated with survival [HR 0.47 (95% CI 0.32-0.69)].

CONCLUSIONS: Desmoplastic melanoma does not share traditional prognostic factors with the melanoma family. Surgical resection with wide margins is needed to optimize survival and routine SLNB may be unnecessary. Furthermore, patients who received adjuvant radiation were at increased risk of dying (HR 1.65) and had decreased OS at 5 years (66% no radiation vs. 50% for adjuvant radiation, P < 0.001). Importantly, none of the traditional prognostic factors for cutaneous melanoma, such as site, Breslow thickness, Clark level, ulceration, and nodal status, had any impact on survival on univariate or multivariate analysis.
 

2. Mixed versus pure variants of desmoplastic melanoma: a genetic and immunohistochemical appraisal. Free Full Text

Miller DD, Emley A, Yang S, Richards JE, Lee JE, Deng A, Hoang MP, Mahalingam M.

Mod Pathol. 2012 Apr;25(4):505-15. Free Full Text

Abstract

Desmoplastic melanoma is subclassified into pure and mixed variants with a higher rate of lymph node metastasis in the latter. Given that reasons for these biological differences are not currently known, we investigated these subtypes with techniques that included genetic and immunohistochemical analyses of 43 cases of desmoplastic melanoma (24 pure, 19 mixed). Direct DNA sequencing was performed on BRAFV600E, RET gene (coding region on exon 11) and KIT (exons 11, 13 and 17). Immunohistochemical stains were performed with antibodies to markers of significance with respect to biological potential of nevomelanocytic proliferations and/or desmoplastic melanoma (Ki-67, CD117, nestin, clusterin, SOX10 and CD271/p75NTR). Polymorphism at the RET coding region (RETp) was noted in 33% of pure (8/24 cases) versus 24% of mixed (4/17 cases); BRAFV600E was absent in all cases of pure (0/24 cases) versus 6% of mixed (1/17 cases); no mutations were found in any of the cases on analyses of exons 11, 13 and 17 of the c-KIT gene (P=NS for all). For immunohistochemical analyses of pure versus mixed: mean percentage of Ki-67 nuclear positivity was 5% (s.d.=5.6) versus 28% (s.d.=12.6, P<0.001); CD117 stained 26% (6/23 cases) versus 78% (14/18 cases, P<0.01); nestin stained 83% (n=19/23 cases) versus 89% (16/18 cases, P=NS); clusterin stained 4% (1/23 cases) versus 6% (1/18 cases, P=NS); SOX10 87% (20/23 cases) versus 94% (17/18 cases, P=NS) and CD271 stained 61% (14/23 cases) versus 67% (12/18 cases, P=NS). Increased CD117 staining in the mixed variant suggests that alterations in the KIT protein may be involved in tumor progression. In addition, the proliferative index of the mixed variant was higher than that of the pure variant.

3.Subclassification of desmoplastic melanoma: pure and mixed variants have significantly different capacities for lymph node metastasis.

George E, McClain SE, Slingluff CL, Polissar NL, Patterson JW.

J Cutan Pathol. 2009 Apr;36(4):425-32.

Abstract

BACKGROUND: There is disagreement about the behavior and optimal management of desmoplastic melanoma (DM), particularly regarding the incidence of lymph node (LN) involvement. Recently, investigators have noted the frequently heterogeneous histologic composition of DM and have found significant differences between pure desmoplastic melanoma (PDM) (>or=90% comprised of histologically typical DM) and mixed desmoplastic melanoma (MDM) [>or=10% DM and >10% conventional melanoma (CM)].

METHOD: We reviewed 87 cases of DM comparing the histologic and clinical features of PDM (n = 44) to MDM (n = 43).

RESULTS: At surgical staging, there were LN metastases in 5 of 23 (22%) MDM patients, whereas all 17 PDM patients had negative LN biopsies (0%) (p = 0.04). PDM was less often clinically pigmented (36% vs. 67%) and had a lower mean mitotic index (1.3 vs. 3.0).

CONCLUSIONS: There are differences between PDM and MDM, the most important of which is the incidence of LN involvement. Our findings support the clinical utility of classifying DM into pure and mixed subtypes because the negligible rate of nodal involvement in PDM does not support the routine performance of sentinel LN biopsy in this subgroup of melanoma patients. In contrast, the incidence of LN involvement in MDM is comparable to that of CM.

4.  Desmoplastic malignant melanoma: a systematic review.

Lens MB, Newton-Bishop JA, Boon AP. Br J Dermatol. 2005 Apr;152(4):673-8.

Abstract

Prompt definitive surgical excision is the treatment of choice for DM. Improved knowledge of the clinical behaviour and histological features of DM is important for more effective management of patients with DM.

5. Desmoplastic melanoma: a review.

Chen LL, Jaimes N, Barker CA, Busam KJ, Marghoob AA.

Abstract:  Desmoplastic melanoma (DM) is a variant of spindle cell melanoma typically found on chronically sun-damaged skin of older individuals. Early diagnosis can be challenging because it is often amelanotic and has a predominantly dermal component. DM can be difficult to diagnose not only clinically but also histologically, and can be mistaken for a variety of benign and malignant nonmelanocytic spindle cell tumors when viewed on prepared histopathology slides. Pathologists have observed that DMs can manifest significant variation with respect to the extent of intratumoral cellularity, fibrosis, and/or perineural invasion. Furthermore, some tumors present with a pure desmoplastic invasive component (>90%) while other tumors display mixed features of DM and nondesmoplastic melanoma. This has led to the separation of DM into 2 histologic subtypes, pure and mixed. With a focus on the distinction between pure and mixed DM, this review will detail what is currently known about the diagnostic features of DM, discuss risk and prognostic factors, and examine the current literature on disease progression and management.

Read More

Talon Zumba

Abstract: 36 yo woman with brown-black macule on heel

HPI:  A 36 year-old woman presented in a panic with a recently discovered dark-brown to black macule on the heel of her left foot.  It had been brought to her attention by her pedicurist.  The patient had consulted the Internet and found articles about acrolentiginous melanoma which worried her greatly. Anamnesis revealed that she had been Zumba dancing a few days before this was discovered.

O/E: The lesion measured eight mm in diameter and was dark brown to black in color.  Dermoscopy revealed a black color with a parallel ridge pattern.

Clinical Photos:

On the suspicion that this represents hemorrhage into the strateum corneum (a condition, when on the foot, called black heel or talon noir) the lesion was pared down with a # 15 blade and some of the dark pigment was easily debrided leaving a skin colored base centrally and some petechael spots were seen at the periphery.  The patient was asked to debride the area gently with a heel shaver andgiven a return appointment in two weeks.

Talon noir can be a frightening entity for a patient.  Lacking a history, a physician can be fooled, since the parallel ridge pattern seen on dermoscopy is also present in acral melanomas.  History trumps clinical appearance,

Follow-up:
At two weeks out, the area is almost completely resolved, this confirming the diagnosis of talon noir.

Two weeks after initial visit.

 

Reference: 

1. Talon Noir(Primary care Dermatology Society)  This is an excellent reference and there is no need for more.

Read More

Complicated Speckled Lentiginous Nevus

Presented by Yoon Cohen, D.O.
Alta Dermatology
Mesa, Arizona

Abstract: An 8-year-old girl with large congenital nevus on the chin, the anterior neck and the right aspect of shoulder and the chest

History: The patient is an 8-year-old girl who was born with congenital nevi on the mid anterior neck and possibly another similar lesion over the right side of the mandible. Over the years, she has developed many 1-3 mm in diagmeter tan papules in and around the chin and submental region. She was seen recently for follow up. Her nevus is slowly maturing but still benign in appearance and there are no outlier lesions in the large defect. Some of the tumor is bilateral.

Clinical photo:

O/E: The skin examination shows a healthy, pleasant child with numerous scattered 1 to 3 mm brown macules and papules in a background of light tan pigmentation on the chin, the anterior neck and the right aspect of the upper chest. There is a well marginated 25 mm in diameter brown patch on the lower mid anterior neck. There is a 3.5 x 4.0 cm hypopigmented patch on the right shoulder

Diagnosis: Complicated Speckled Lentiginous Nevus (SLN)

Questions: Please share your experiences in managing patients with SLN similar conditions.  Do you feel there is a concern that this lesion may undergo malignant transformation.  What do you think of the hypopigmented area?

Discussion: A speckled lentiginous nevus (SLN, Nevus Spilus) can be a congenital or acquired pigmented lesion. More recently arguments are in favor of speckled lentiginous nevi as subtype of congenital nevi including the following observations: frequent presence at birth or noted soon therafter; patterns of distribution reflecting embryonic development; hamartomatous behavior with various types of nevi; and histologic features of congenital melanocytic nevi within the lesions. There are two types of speckled lentiginous nevus -- Nevus spilus maculosus and nevus spilus papulosus. The macular type is characterized by dark speckles that are completely flat and rather evenly distributed on a light brown background, resembling a polka-dot pattern. In contrast, nevus spilus papulosus is defined by dark papules that are of different sizes and rather unevenly distributed, reminiscent of a star map. Perhaps, the most pressing concern is its potential malignant transformation. The propensity to develop Spitz nevi appears to be the same in both types of speckled lentiginous nevus whereas the development of malignant melanoma has been reported far more commonly in nevus spilus maculosus. Torchia and her colleague argue that malignant transformation of melanocytes might occur in SNL not because the hyperpigmented background features any specific procarcinogenic abnormality but because there are more melanocytes with SNL, yielding a slightly higher likelihood of developing any type of melanomcytic lesion. It is our best interest that we monitor our patients with SNL pediodically to detect any early signs of malignant transformation.

References:
1. Happle R. Speckled lentiginous naevus: which of the two disorders do you mean? Clin Exp Dermatol. 2008;34:133-135
2. Paraskevas LR, Halpern AC, Marghoob AA. Utility of the Wood's light: five cases from a pigmented lesion clinic. Br J Dermatol. 2005 May; 152(5): 1039-44
3. Menon K. Dusza SW, Marghoob AA, et al. Classification and prevalence of pigmented lesions in patients with total body photographs at high risk of developing melanoma. J Cutan Med Surg. 2006 Mar-Apr; 10(2):85-91
4. Schaffer J. Orlow S, Lazova R, et al. Speckled Lentiginous Nevus Within the Spectrum of Congenital Melanocytic Nevi. Arch Dermatol. 2001; 137:172-78
5. Torchia D. Schachner L. Is speckled lentiginous nevus really prone to dysplasia/neoplasia? Pediatr Dermatol. 2012 Jul-Aug; 29(4):546-7

Comments:
1. Dr. Ashfaq Marghoob: 
These patients (SLN of maculosa type) are at slightly higher risk for developing MM. Continued surveillance for focal change is what I do for these patients.

2. Dr. Rudolph Happle:
Thank you very much for letting me see this interesting case! Apparently, this is a classical example of papular SLN (papular nevus spilus).  Why is your diagnosis "Complicated SLN"? I would call this a complicated case if a melanoma would develop, but the risk is rather low because It's not a macular nevus spilus.

3. Dr. Harper Price: 

Thank you for sharing this interesting case.  It does look very classic for a SLN.

The risk of malignant transformation is very low and likely starts at puberty so I would follow her yearly and if there was a lot of change, even more frequently and use photography to help.  These SLN can have many other nevi within in as stated, including Spitz and blue nevi.

Hers looks quite good and not worrisome.  The area of hypopigmentation, if new, may represent vitiligo or if present early on, a hypochromic nevis (“twin spotting”).

These SNL certainly carry a much lower risk than a typical congenital melanocytic nevus of this size for melanoma development. 
Lifelong follow-up is my recommendation

Read More

Melanoma Does Not Read Textbooks I

Presented by Yoon Cohen, D.O. and David Elpern, M.D.

Abstract:  67-year-old man with a 3-month history of pigmented papules on the face and torso.

HPI: The patient is a 67-year-old man with a 3-month history of pigmented papules on the face and torso. He has past medical history of enucleation over 19 years ago for ocular melanoma. Since enucleation, he has been fairly healthy and staying active with his life. He has not noticed any recent weight loss, fatigue, and other associated constitutional symptoms.

O/E: The skin examination showed a pleasant and outgoing man with ~ 50 well defined 2-4 mm multiple scattered purple to blue-black firm papules on the face and torso. No lymphadenopathy noted. 

Clinical Pictures: 

Purple to blue-black firm papules on the cheek

Several scattered pigmented papules on the upper back

Dermoscopic View

Pathology: A dermal nodule of atypical, focally pigmented epithelioid cells exhibiting large nucleoli and focal tumor necrosis infiltrating the dermis and skeletal muscle consistent with metastatic malignant melanoma. No junctional component is present. Immunoperoxidase staining reveals the tumor cells to be positive for Mart-1/Melan-A, S100 and HMB45, confirming the histologic diagnosis.

Lab: CBC abd chemistries are normal. LDH is borderline high but not above normal range.

Imaging Studies: 

1. Abdominal Ultrasound: There are numerous heterogeneously hyperechoic masses within the liver, most compatible with metastatic disease. The largest of these is within the right hepatic lobe and measures up to 6.6 cm in greatest diameter. 

2. Chest PA/Lateral Xrays: No pulmonary nodules or pleural effusions are evident.

Diagnosis: Metastatic melanoma. Most likely ocular melanoma metastasis to the liver.

Discussion: 

This patient poses some therapeutic question.  His melanomas do not bother him at this point and he keeps saying that he feels fine.  He's an avid golfer.  If he gets plugged into the system and loses this season; can we promise him another season in 2014?  He is a simple man; retired, no outside source of income. His melanoma, dormant for 19 years is suddenly active again. Why?

Question:

Knowing chemotherapy may significantly affect his energy level and he may experience other significant side effects, we are not sure what would be the best option for this patient at this point. We'd appreciate you sharing your similar experiences with your patients.

Follow-up: Many attempts were made to contact the patient and get him back for a follow-up discussion; but he never answered his phone or returned letters.  He died ~ 5 months later.  We hope he payed a few rounds of golf over the summer and suspect that his life was easier without futile attempts at palliation.

References:
1. Progression of ocular melanoma metastasis to the liver: the 2012 Zimmerman lecture.
JAMA Ophthalmol. 2013 Apr;131(4):462-9. 

Grossniklaus HE.

Source

L. F. Montgomery Laboratory, Emory Eye Center, Atlanta, GA 30322, USA. ophtheg@emory.edu

Abstract

IMPORTANCE:

To the best of my knowledge, this study demonstrates for the first time small, apparently dormant micrometastasis in the liver of patients with uveal melanoma.

OBJECTIVE:

To evaluate the histological and immunohistochemical findings in metastatic uveal melanoma to the liver.

DESIGN:

Samples of liver were obtained at autopsy from patients who died of metastatic uveal melanoma to the liver.

SETTING:

L. F. Montgomery Laboratory, Emory Eye Center, Atlanta, Georgia.

PARTICIPANTS:

A total of 10 patients who died of metastatic uveal melanoma to the liver.

INTERVENTION:

Sections of the liver were examined with hematoxylin-eosin, periodic acid-Schiff, Masson trichrome, or reticulin stains.

MAIN OUTCOME MEASURES:

The tumors' morphology, growth pattern, mean vascular density, and mitotic index were determined with the aid of immunohistochemical stains for S100, HMB45, CD31, and Ki67.

RESULTS:

Stage 1 metastases (defined as tumor clusters ≤50 μm in diameter) were identified in the sinusoidal spaces of 9 of 10 patients (90%). Stage 1 metastases were avascular and lacked mitotic activity. Stage 2 metastases (defined as tumors measuring 51-500 μm in diameter) and stage 3 metastases (defined as tumors measuring >500 μm in diameter) were found in all patients. Immunohistochemical stains were positive for S100 or HMB45 in all tumors. Overall, stage 1 metastases outnumbered stage 2 metastases (which outnumbered stage 3 metastases). The mean vascular density and mitotic index increased from stage 2 to stage 3 metastases (P < .05). The architecture of stage 2 metastases mimicked the surrounding hepatic parenchyma, whereas stage 3 metastases exhibited either lobular or portal growth patterns.

CONCLUSIONS:

Uveal melanoma that spreads to the liver can be categorized as stage 1 (≤50 μm in diameter), stage 2 (51-500 μm in diameter), or stage 3 (>500 μm in diameter) metastases. The later stage exhibits a lobular or portal pattern of growth. During this progression, tumors become vascularized and mitotically active.

2. Domancy of Metastatic Melanoma

Pigment Cell Melanoma Res. 2010 Feb;23(1):41-56. 

Ossowski L, Aguirre-Ghiso JA.

Source

Division of Hematology and Oncology, Department of Medicine, Mount Sinai School of Medicine, New York, NY, USA. liliana.ossowski@mssm.edu

Abstract

Metastatic dormancy of melanoma has not received sufficient attention, most likely because once detectable, metastasis is almost invariably fatal and, understandably, the focus has been on finding ways to prolong life of patients with overt recurrences. Nevertheless, analysis of the published clinical and experimental data on melanoma indicates that some aspect of melanoma biology imitate traits recently associated with dormancy in other solid cancers. Among them the ability of some melanomas to disseminate early during primary tumor progression and once disseminated, to remain undetected (dormant) for years. Comparison of cutaneous and uveal melanoma indicates that, in spite of being of the same origin, they differ profoundly in their clinical progression. Importantly for this discussion, between 40 and 50% of uveal melanoma remain undetected for longer than a decade, while less than 5% of cutaneous melanoma show this behavior. Both types of melanoma have activating oncogene mutations that provide autonomous pro-proliferative signals, yet the consensus is that those are not sufficient for tumor progression. If that is the case, it is possible to envision that signals from outside the tumor cell, (microenvironment) shape the fate of an individual disseminated cell, regardless of an oncogene mutation, to progress or to pause in a state of dormancy. To stimulate further debate and inquiry we describe here a few examples of potential signals that might modify the fate of disseminated cell and provide brief description of the current knowledge on dormancy in other cancers. Our hope is to convince the reader that disseminated melanoma cells do enter periods of prolonged dormancy and that finding ways to induce it, or to prolong it, might mean an extension of symptoms-free life for melanoma patients. Ultimately, understanding the biology of dormancy and the mechanisms of dormant cell survival, might allow for their specific targeting and elimination.

Read More

Incidentaloma

The patient is an 82 man who presented  for evaluation of a facial lesion.  That lesion was a basal cell carcinoma.  However a complete skin exam revealed a suspicious tumor on the right upper back. 

The patient has type III skin.  This "new" lesion on the back is an eight mm in diameter hypopigmented papule with an eccentrically placed  2 mm pigmented papule.

Clinical Photos:

Polarized View

Non-Polarized View

The Power of BLINK:
B = not benign
L = Lonely  1 point
I = Irregular pigment 1 point
N = (pt. unaware of lesion)
K = Known Dermatoscopic abnormalities  1 point
Score = 3  (Mandates biopsy)

The patient is scheduled for an excisional biopsy of the back lesion.  What are your thoughts at this time?

Biopsy shows this to be Malignant Melanoma
Type: unclassified
Thickness: 2.80 mm
Margins: signs of regression at ;ateral margin
Ulceration: absent
Mitoses: > 1/ mm squared
Vascular invasion: absent
Precursor lesion absent

Read More

Amelanotic Acral Lentiginous Melanoma

Abstract: 61 yo man with 4 - 5 year hx of a tumor on foot.

HPI: The patient is a healthy 61 year old man with a 4 - 5 year history of a slowly growing lesion on the plantar aspect of his right foot. On a recent trip to Jamaica it bled, leading him to consult a podiatrist who astutely did a biopsy. The patient has sarcoidosis which has been treated with weekly i.m. methotrexate for the past two years. (I do not know the dose byt presume it is around 15 mg).

O/E: 2 x 1 cm flesh-colored nodule. Crust in photo is from punch biopsy. Remainder of cutaneous exam unremarkable. No palpable regional lymph nodes. Dermatoscopic exam was not rewarding.

Photo:

Dermoscopic Images:

Lab: Mild leucopenia 3700. Otherwise all chemistries and LDH normal

Pathology: ALM 3.68 (at least) mm thick, (at least) Level IV.
Tumor thickness may be deeper tumor is present at the base of the specimen.
Regression: Not Present
Vascular/lymphatic invasion: Not identified
Mitotic Activity: 7/10 HPF
Tumor Infiltrating Lymphocytes: Non-brisk
Vertical Growth Phase: Present

Discussion: Although this tumor is called "acral lentiginous melanoma" it clearly is a nodular lesion. Might it better be called "acral nodular melanoma?" The patient will need staging and the, depending on findings of staging studies, a wide-local excision with lymph node mapping . He is being referred to the melanoma clinic at Dartmouth Mary Hitchcock Medical Center.

This is an amelanotic acral melanoma that has been present 4 - 5 years by history. Amelanotic acral melanoma are scary lesions as clinically and dermoscopically they do not appear to be worrisome.
It is well-recognized that these can fool practitioners, as they are only rarely seen even by dermatologists and a high index of suspicion is needed. The podiatrist who saw the patient was astute to biopsy the lesion on his first visit.

References:
1. Acral lentiginous melanoma: a clinicoprognostic study of 126 cases.
Phan A, Touzet S, Dalle S, Ronger-Savlé S, Balme B, Thomas L.
Br J Dermatol. 2006 Sep;155(3):561-9.
Department of Dermatology, Hôtel Dieu, Claude Bernard University, 69288 Lyon cedex 02, France.
Abstract:
BACKGROUND: Although the histopathological subtype of melanoma has not been clearly proven to carry independent prognostic significance, acral lentiginous melanoma (ALM) seems to confer a poorer prognosis mainly because disease is often more advanced at the time of diagnosis.
OBJECTIVES: To investigate the distinctive epidemiological and clinical characteristics of ALM, a peculiar histological entity, and to identify prognostic factors.
METHODS: We performed a register-based review of cases from a single large referral centre, the University Hospital Department of Dermatology, Lyons, France. We reviewed patient demographics, the initial presentation of the lesion, and clinical outcome. ALM-specific and disease-free survival were estimated using the KaplanMeier method and compared using the log-rank test. A Cox model was used to identify prognostic factors.
RESULTS: One hundred and twenty-six patients were identified as having histopathology-proven ALM in our melanoma patient register from 1996 to 2004. There were 46 (37%) subungual ALM and 80 (63%) ALM on soles, palms and nonvolar sites. The mean age at diagnosis was 63 years. There were 44 (35%) men and 82 (65%) women, sex ratio M/F 1 : 1.86. The mean Breslow thickness was 2.51 mm (range: in situ to 20 mm). There was no evidence of overexposure to ultraviolet radiation, nor was there found a predisposing genetic trait. Only 16 (13%) patients recalled a history of trauma. Thirty-four ALM (28%) were unpigmented. The median ALM-specific and disease-free survival were 13.5 and 10.1 years, respectively. The 5-year survival rate was 76%. Multivariate analysis identified tumour thickness, male gender and amelanosis as independent clinical prognostic factors for both ALM-specific and disease-free survival.
CONCLUSIONS: Our study provides specific information on the clinical characteristics and outcome of this uncommon histological subtype of melanoma. However, the pathogenesis remains unknown. Breslow thickness, male gender and amelanosis were significantly associated with a poorer prognosis.

2. Acral lentiginous melanoma mimicking benign disease: the Emory experience.
Soon SL, Solomon AR Jr, Papadopoulos D, Murray DR, McAlpine B, Washington CV.
J Am Acad Dermatol. 2003 Feb;48(2):183-8.
Abstract
BACKGROUND: Plantar and subungual melanoma exhibits a higher misdiagnosis rate relative to other anatomic sites. Misdiagnosis and delay in diagnosis are statistically associated with poorer patient outcome. Awareness of atypical presentations of acral melanoma may, thus, be important to decrease misdiagnosis rates and improve patient outcome.
METHODS: We conducted a retrospective case review of plantar or lower-extremity subungual melanoma performed at Winship Cancer Center, a tertiary care, referral center affiliated with Emory University, between 1985 and 2001.
RESULTS: A total of 53 cases of plantar or lower-extremity subungual melanoma were identified. Of 53 cases with a final diagnosis of melanoma, 18 were initially misdiagnosed. Misdiagnoses included wart, callous, fungal disorder, foreign body, crusty lesion, sweat gland condition, blister, nonhealing wound, mole, keratoacanthoma, subungual hematoma, onychomycosis, ingrown toenail, and defective/infected toenail. Of the 18 misdiagnosed cases, 9 were clinically amelanotic.
CONCLUSION: Awareness that amelanotic variants of acral melanoma may assume the morphology of benign hyperkeratotic dermatoses may increase the rate of correct diagnosis and improve patient outcome.

Read More

The Power of BLINCK

Presented by Yoon Cohen MS IV, University of New England, Biddeford, Maine and David Elpern MD, Williamstown, Massachusetts

Abstract: 68 yo woman with 4-6 months history of an atypical melanocytic lesion.

HPI: This healthy 68 yo woman with type II skin presented to the clinic with 4-6 months history of an atypical melanocytic lesion on the left knee. She had noticed an increase in size and change in color and was concerned about these changes in the lesion.

O/E: There was a 7 mm in a diameter asymmetrical brownish macule on the left knee. The lesion showed an irregular border with varied colors.

Clinical photographs:

Dermoscopic images:

Microscopic images:

Dermatopathology report:

The specimen exhibits a proliferation of moderate to severely atypical melanocytes distribubted in irregular nests, as well as singly at and above the dermal epidermal junction, with pagetoid spread to the granular layer and near confluence over at least three rete ridges. These findings support the histologic diagnosis of melanoma-in-situ.

Our appreciation to Dr. Deon Wolpowitz, MD from Boston Univeresity, Dermatopathology, for providing these photomicrographs for the case.

4X

10X

20X

20X

Diagnosis:

Malignant melanoma in situ

Discussion:

The BLINCK approach:

We followed the BLINCK checklist, introduced by Dr. Peter Bourne, a founder of the Skin Cancer College of Australia and New Zealand (SCCANZ). The score for the lesion was added up to 4 by criteria as following.

1. B. The lesion was not clearly benign at our first initial evaluation

2. L. The lesion appeared to be lonely without any other similar melanocytic lesion near by

3. I. The lesion appeared to be irregular outline and color on our dermoscopic exam

4. N & C. The patient was nervous about the change in color in past 4-6 months

5. K. The lesion exhibited known clues when viewed with a dermatoscope. See "Chaos and Clues" reference below.
BLINCK Score = 4

According to the BLINCK approach, a lesion should be biopsied if the BLINCK score is 2 or more out of a possible 4. Therefore, the we excised this lesion and sent for a pathologic evaluation.

According to Dr. Bourne, the BLINCK approach is presented as a simple method to assist the clinician with the decision of whether to biopsy a skin lesion or not. The use of this algorithm will improve the pickup rate of potentially serious skin cancers as well as reduce the number of unnecessary benign lesion excisions. BLINCK may be especially helpful to clinicians who have only basic or intermediate dermoscopy skills but who are regularly called upon to assess skin lesions in their practices.

Questions:

1. Would you consider using the BLINCK approach at your practice?

2. If you already have adapted the BLINCK approach, how have your experiences been?

References:

1. McColl I. BLINCK. http://idsblinck.blogspot.com/2009/11/blinck.html. Updated November 19, 2009. Accessed September 7, 2010

2. Rosendahl C, Kittler H, Cameron A, et al. CHAOS & CLUES - The Algorithm. http://www.chaosandclues.blogspot.com. Updated November 19, 2009. Accessed September 7, 2010

Read More