5/23/2017 Disclosure of Relevant Financial Relationships Flank mass in a 43-year-old male USCAP requires that all faculty in a position to influence or control the content of CME disclose any relevant financial relationship WITH COMMERCIAL INTERESTS which they or their Bobbi S. Pritt, MD, MSc Mayo Clinic spouse/partner have, or have had, within the past 12 months, which relates to the content of this educational activity and creates a conflict of interest. Dr. Bobbi Pritt declares she has no conflicts of interest to disclose. History • 43 year old male presented with tender plaques and nodules involving his left flank. • Patient is from the upper Midwestern United States • No travel outside of the United States. • His previous medical history is significant for well-controlled insulin-dependent diabetes. • An excisional biopsy was performed of one of the nodules H&E, 20x total magnification H&E, 40x total magnification H&E, 100x total magnification H&E, 200x total magnification 1 5/23/2017 H&E, 100x total magnification H&E, 100x total magnification H&E, 100x total magnification Masson’s Trichrome, 40x total magnification Diagnosis? A. B. C. D. E. Helminth, possible roundworm. Helminth, possible cestode Not a helminth, foreign body Not a helminth, fat necrosis No idea, (ask a colleague!) Masson’s Trichrome, 100x total magnification 2 5/23/2017 Lipomembranous Fat Necrosis - General • A.k.a. membranocystic fat necrosis, lipomembranous panniculitis • Initially described in 1974 in bone and subcutaneous tissues in a case of sudanophilic leukodystrophy of cerebral hemispheres • Commonly observed in association with venous insufficiency, arterial ischemia, diabetes, erythema nodosum, and lupus • Degenerative process involving mature adipose tissue • Described from various sites: subcutaneous tissue (usually lower extremities), breast, testicle, bone marrow, benign and malignant tumors • Observed in 0.7% to 9% of inflammatory lesions of subcutaneous tissues • More common in women (89% in one case series: Snow et al.) • Mean age 57 years (range 32-80 years) Lipomembranous Fat Necrosis - Morphology • Cystic (most common; often microcystic) and non-cystic forms • Adipocyte necrosis • Formation of eosinophilic anucleate, crenated, undulating ribbon-like membranes. • Pseudopapillary tufts • PAS-positive, diastase resistant membranes • Cysts may form complex “Arabesque” patterns CD68 and lysozyme positive Polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy (courtesy of Dr. Andrew Folpe) • Autosomal recessive genetic disorder • Associated with polymorphisms of 2 genes: TYROBP (DAP12) and TREM2 • Characterized by fractures (resulting from polycystic osseous lesions), frontal lobe syndrome, and progressive pre-senile dementia beginning in the fourth decade. • Patient: 34 year-old female residing in a skilled nursing facility. Presented with fractures of the tibia and other bones. Image courtesy of Lawrence Gibson, MD 3 5/23/2017 Large Worms in Subcutaneous Tissue • Roundworms DDX: Worms in Tissue • Dirofilaria species (size in cross-section: 300-350 µm) • Onchocerca volvulus (150-400 µm diameter) • Loa loa (~500 µm in diameter) • Tapeworms • Taenia solium (cysticercosis) • Other Taenia sp. (coenurosis) • Spirometra sp. (sparganosis) Examples of large roundworms Anatomy of a Roundworm Zoonotic Onchocerca species Dirofilaria species • Cuticle (including spines, ridges) • Well-defined musculature • Internal organs Image by Lars Westblade, PhD Image by Ryan Relich, PhD 4 5/23/2017 Anatomy of a tapeworm larva • Thin acellular cuticle • Smooth muscle fibers • Calcareous corpuscles (small calcified bodies) • Depending on the tapeworm: protoscoleces (immature heads) with suckers and hooklets, fluidfilled sac A recent case of lipomembranous fat necrosis simulating a cestode. Lipomembranous fat necrosis Sparganosis Other examples of tapeworm larvae A recent case of cysticercosis Image by Paul Valenstein, MD 5 5/23/2017 Approach DDx: plant material • Measure! 4 mm 1 mm Approach Approach • Measure! • Look for defined structures • Measure! • Look for defined structures • Cuticle and underlying musculature LM fat necrosis No well-defined cuticle (external ‘lining’ merges with underlying fibrous connective tissue • • • • Cuticle and underlying musculature Reproductive or gastrointestinal organs Eggs (requires familiarity) Round worm Approach • Measure! • Look for defined structures • • • • Cuticle and underlying musculature Reproductive or gastrointestinal organs Eggs (requires familiarity) • Special stains • Masson’s trichrome, PAS, acid fast • Polarizable? • Consult (e.g. microbiologist, colleague, CDC ID Path and DPDx groups) References • Nasu et al. A lipid metabolic disease—“membranous lip dystrophy”—an autopsy case demonstrating numerous peculiar membrane-structures composed of compound lipid in bone and bone marrow and various adipose tissues. Acta Pathol Jpn 1973;23:. 539–558 • Segura S, Pujol RM. Lipomembranous fat necrosis of the subcutaneous tissue. Dermatol Clin 2008;26:509-517. • Snow JL, Su WPD. Lipomembranous (membranocystic) fat necrosis: clinicopathologic correlation of 38 cases. Am J Dermatopathol 1996;18(2):151-155. • Diaz-Cascajo C, Borghi S. Subcutaneous pseudomembranous fat necrosis: new observations. J Cutan Pathol 2002;29:5-10. • Pritt BS, Elhosseiny A. Artifacts and pitfalls in infectious disease pathology. In Pathology of Infectious Diseases, Procop GW and Pritt BS (Eds). 2015 Elsevier: Philadelphia, PA 6 5/23/2017 7
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