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Dysplastic Nevus with Severe Atypia” Melanocytic conundrums Ron Rapini MD, Chernosky Chair, Dept Derm Professor of Pathology Univ Texas Medical School at Houston MD Anderson Cancer Center Conflict of interest statement: None - I have no stock in grocery stores that sell Rapini By now you’re probably sick of the relentless attack of the alien melanocytes DAILY in your practice Melanocytes come from the neural crest – born to wander – “wunderlust” If you think about it, there are not too many tumors 1 mm in size which regularly metastasize Main goal of the dermatopathologist should be to be helpful; even though often we don’t know for sure what all these melanocytic neoplasms are… "Be kinder than necessary, for everyone you meet is fighting some kind of battle." Plastic surgeon: “I am sorry; I just need a definitive diagnosis so I can know what to do. .” (complaining about report “atypical melanocytic proliferation – atypical nevus favored over melanoma”) “Morphological diagnosis, whether of birds, fish, plants, or pathological processes in human beings, is 100% subjective” Ackerman AB. Discordance among expert pathologists in diagnosis of melanocytic neoplasms. Hum. Pathol. 1996; 27; 1115-1116. Really, H&E stain is primitive approach of using pink and blue dyes to predict complex biological behavior • Future probably lies with more sophisticated methods like FISH, CGH, etc, but many feel that these do not yet give definitive diagnoses in some cases We, the H&E Olympians attempt to predict biologic behavior from pink and blue splotches What is the predicated biologic behavior of this person? Predictive clues? 1. Texas shirt 2. Orange color 3. Shorts 4. Weird hat 5. Beer in hand 6. Socks pulled up 7. Long hair Homerun hitters vs Base hitters • Homerun hitter dermpaths (one diagnosis – melanoma - most of the time) are either really really right or really really wrong • Base hitter dermpaths (differential all the time) are almost always right, but often not much help • The ART of pathology is to be a homerun hitter as much as possible but to go for the base hit when necessary Five dermatopathologists have seen this slide… • Three think that it is benign and two favor malignancy • They can have their opinion, but definitive answer may be unknown Vague terms (“The favorite plant of radiologists and pathologists is the hedge”) • Consistent with • Suggestive of • Near the margin • Narrow margin • Approximates the margin Clues – Ackerman published several books on various clues in dermpath • Our diagnoses and predictions are based upon various clues or other findings • Not all clues are valid: I prefer not to use algorithms for that reason (blind alley if rely too heavily on just one finding) • I prefer to look at multiple findings in tandem “Our criteria may be wrong, but at least we have criteria” • Better than using “feeling in your gut” • Not all criteria have equal sensitivity and specificity • Use multiple criteria So let’s start with a lawsuit! Happened to me when 30 years out of medical school and 25 years doing dermpath • The celebration: my first “Dear Dr Rapini” letter received 19 year old construction worker, shoulder “Dysplastic nevus with severe atypia” I stated the features: • Atypical epithelioid cells in superficial portion, with junctional mitoses, pagetoid • Congenital features, banal single filing • Clefts, Spitz nevus features, maturation • No ulceration, nearly no inflammation “Dysplastic nevus with severe atypia” My concluding statement: • Does not meet enough criteria for melanoma • If this WERE a melanoma, depth would be no greater than 0.5 mm • Melanocytes extend more deeply than that but those appear benign • Conservative re-excision recommended Patient went to plastic surgeon and had re-excision • He decided to take 3 mm margins • Tissue only showed scar • Patient never had local recurrence • Nothing happened until 5 years later when he developed the node in neck Lawsuit said: • 2002: You misread tissue as a BENIGN dysplastic nevus with SEVERE atypia. You recommended excision of margins. • 2007: Mass in lymph node on neck They sued four parties 1. Ron Rapini MD, read original 2. Pathologist who read the re-excision as negative (he was right) 3. Path Lab that employed that 2nd pathologist 4. Lab Corp that contracted with that lab Dermatologist wrote in chart: “seriousness of the diagnosis explained to patient”, before sending him to plastic surgeon After the metastasis occurred with no local recurrence, another dermatologist wrote “obviously the lesion metastasized before Dr Rapini ever read the slides” Slides reviewed elsewhere after metastasis known • “Nevoid malignant melanoma” • “Considerable atypia in upper portion but pronounced maturation” • “Sufficient atypia in upper portion to point to melanoma” • “Overall pattern deceptive, however” • Nevoid portion extends to deep margin at 1.5 mm (level IV) (I had only measured the atypical portion at 0.5 mm) Patient alive, had large node removed, no further recurrence yet after 2 yrs • Plaintiff dermpath expert and oncologist: “Case should have been sent out for consult and definitive diagnosis Patient was denied the sentinel lymph node biopsy and interferon which would have made a difference Margins would have been wider.” Mediator said (in private) We attorneys just go out and hire whores, because they are more predictable and controllable Final result: All four original parties were dismissed So . I can talk about it They then sued a 5th party, the university, which paid them $60K Attorney for university said that this is a teenager case, and don’t like child cases: “cannot predict what jury will do” If 80 year old, then would not have settled Preventive tactics • Biopsy everything • Call everything malignant • Hedge all the time and don’t give specific answer • “Clinical correlation needed” • “Re-excision needed” • Order unnecessary preventive tests and consult others frequently Add sentence: • “Melanoma was considered, and was not favored, but cannot be completely excluded” Masterful hedging? Worthless? Takes more guts to call something benign rather than malignant • Only bad things happen to the pathologist and the patient if the malignant lesion was called benign • If benign called malignant, no one will know, they will remain happy there was no recurrence, and likely no one will sue for “over treatment” Hence, we have . • Sebaceous adenomas in the setting of Muir-Torre syndrome are all sebaceous carcinoma • Proliferating pilar cysts are all SCC If you live in paranoia . • Your diagnoses become less specific • More atypia, more malignancy • LONGER notes, longer reports Personally, I still do things pretty much the same way Internal or External Consultations help protect you • But they are overrated • Bandwagon opinion of “the group” • Often everyone just hedges • If someone does NOT hedge, how do you know they are right? • Keep a record (in house, use initials?) I testified in this lawsuit: Pathologist diagnosis on solitary single papule: “histiocytic proliferation – re-excision recommended” What would you do if you were the plastic surgeon? “Histiocytic disorder – re-excision recommended” Plastic surgeon did nothing Lesion metastasizes (turns out the “histiocytes” were melanocytes) Patient sues plastic surgeon, not pathologist “Histiocytic disorder – re-excision recommended” Plastic surgeon testifies he did not re- excise because this pathologist ALWAYS says “re-excision recommended,” so it has no meaning Pathologist testifies: “Yes, I do say on ALL skin biopsy reports with incomplete margins, re-excision recommended” Case goes to court, jury finds doctor not at fault Histiocytic proliferation - case lessons • “Re-excision recommended” is pathologist putting the onus on the clinician • Clinician almost obligated to do it, but if you don’t, should write explanation of why not in the chart • Make sure patient is informed, give copy of pathology report, and document OK, with that introduction (and with the knowledge that lawyers are lurking, we are ready to discuss: DYSPLASTIC NEVI! First of all – we cannot agree on what to call it Dysplastic nevus (DN) Nevus with architectural disorder (NAD, NWAD) Clark’s nevus Active nevus Atypical nevus Atypical mole And we have invented many other terms for the grey zone lesions • MELTUMP –mel tumor uncertain malig potential • SAMPUS – superficial atyp mel prolif uncertain signif • SIMP – sun-induced mel prolif • AMP – Atyp mel prolif • AST – Atyp Spitz tumor The concept of dysplastic nevus is very controversial: induces spasms in many dermatopathologists Ackerman AB. Histopathology. 1988 Sep;13(3):241- 256. What naevus is dysplastic, a syndrome and the commonest precursor of malignant melanoma? A riddle and an answer. Dysplastic nevus = Clark’s nevus = “active nevus” • 5% or 50% of population – criteria vary! • More significant if +FH melanoma, multiple atypical nevi • “Most common nevus in man” - Ackerman • “Growth industry for derm” - Clark Dysplastic nevus • NIH consensus conference (JAMA 1992): Clinician should call them “atypical moles”, pathologist should call them “nevus with architectural disorder” and should grade the cytology “mild, moderate, severe” My 3 favorite things to distinguish melanoma from dysplastic nevus (but exceptions to all of this) 1. More pagetoid 2. More atypia 3. More lymphocytes (“smart bombs”) (Ackerman’s favorite was asymmetry, I think, but flat dysplastic nevi don’t have enough dermal component to evaluate that) Pagetoid melanocytes (Ackerman calls “scatter”) • Melanoma • Spitz nevus, pigmented spindle cell nevus
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