Primary cutaneous histiocyte and rich CD30+/CD56+ anaplastic large of the scalp with prominent angio- and neuroinvasion L. Boudova, D.V. Kazakov, P. MukenÉnabl, V. Kuntscher and M. Michal

Age: 57 years Sex: F monoclonal rearrangement of T-cell receptor gene gamma and absence of DNA of EBV were Clinical features: The patient consulted a neurolo- detected. gist because of headache. During the next three J Fig. 1: A non-ulcerated part of the scalp shows months a tumour spread rapidly over the whole a vaguely nodular, pale tumour infiltrate in the right half of her neurocranium, showing vast dermis with remnants of hair follicles (HE). skin ulcerations, purulent secretion, massive soft J Fig. 2: Geographic necroses in deeper parts of tissue destruction, and incipient bone involve- the tumour (HE). ment. J Fig. 3: Few large anaplastic cells, some resem- Diagnosis: Primary cutaneous histiocyte and bling Reed-Sternberg cells, in a background neutrophil rich CD30+/CD56+ anaplastic large of plentiful histiocytes, , and cell lymphoma (ALCL). small (HE). Follow-up: The immunocompetent patient was J Fig. 4: Histiocytes are the prevailing compo- administered six cycles of chemotherapy nent (CD68). (CHOP), and proceeded to high-dose chemo- J Fig. 5: CD30+/CD56+ neoplastic cells scat- therapy with autologous peripheral stem tered individually or in small groups: CD30 cell transplantation as a remission consolida- (a), CD56 (b). tion. She has remained in complete remission J Fig. 6: Invasion of blood vessels (a) and for more than six years. nerves (b). The neoplastic cells within the vascular walls and nerves were also Comment: Primary cutaneous neutrophil-rich CD30+CD56+ (not shown). ALCL is very rare. Occurring predominantly on the head, it is characterized histologically by a References large number of neutrophils which may obscure the anaplastic or pleomorphic tumour cells. Ini- Boudova L, Kazakov DV, Jindra P, Sima R, Vanecek T, tially thought to be associated with HIV infec- Kuntscher V, Vozobulova V, Bouda J, Michal M (2005) tion, it has also been found in immunocompe- Primary cutaneous histiocyte and neutrophil rich CD30+ and CD56+ anaplastic large cell lymphoma with tent patients. The dramatic presentation with a prominent angioinvasion and nerve involvement in the rapid growth, purulent discharge (ªpyogenic forehead and scalp of an immunocompetent woman. J lymphomaº), widespread tissue destruction, but Cutan Pathol, accepted for publication Aug 2005 an excellent prognosis seems to be typical. Burg G, Kempf W, Kazakov DV, Dummer R, Frosch PJ, The presented case carries a remarkable com- Lange-Ionescu S, Nishikawa T, Kadin ME (2003) Pyo- genic lymphoma of the skin: a peculiar variant of pri- bination of clinicopathological features. Clini- mary cutaneous neutrophil-rich CD30+ anaplastic cally, the tumour infiltration was very extensive large-cell lymphoma. Clinicopathological study of four and deep. Morphologically, this tumour is not cases and review of the literature. Br J Dermatol only rich in neutrophils, but histiocytes prevail 148(3):580±586 Jhala DN, Medeiros LJ, Lopez-Terrada D, Jhala NC, Krish- in the background. Angioinvasion and nerve in- nan B, Shahab I (2000) Neutrophil-rich anaplastic large filtration are striking. The neoplastic cells coex- cell lymphoma of T-cell lineage. A report of two cases press CD30 and CD56, a rare feature represent- arising in HIV-positive patients. Am J Clin Pathol ing a dilemma for a precise classification. On im- 114(3):478±482 munohistochemistry, the majority of neoplastic Mann KP, Hall B, Kamino H, Borowitz MJ, Ratech H (1995) Neutrophil-rich, Ki-1-positive anaplastic large-cell ma- cells are also positive for CD7, CD45RO, CD8, lignant lymphoma. Am J Surg Pathol 19:407±416 granzyme B, TIA-1, and perforin, a few tumour Natkunam Y, Warnke RA, Haghighi B, Su LD, LeBoit PE, elements express also CD5 and CD43, while Kim YH, Kohler S (2000). Co-expression of CD56 and ALK1 kinase, epithelial membrane , Ep- CD30 in with primary presentation in the skin: clinicopathologic, immunohistochemical and mo- stein-Barr virus (EBV; LMP), CD57, CD138, lecular analyses of seven cases. J Cutan Pathol 27: 392± CD20 and CD79a are negative. Using PCR a 399 a Primary cutaneous histiocyte and neutrophil rich CD30+/CD56+ anaplastic large cell lymphoma J 25