H am artom atous Basal-C ell C arcinom a D O U G L A S G. H A M IL T O N , M .D ., A N D A R T H U R P. Z E I T L I N , M .D.
A 25-year-old w om an d e v e lo p e d a b asa l-eell carcin om a In the skin o ve r th e right sca p u la . The liistop ath olog y o f the lesion r e v e a le d elem en ts o f ty p ica l b asa l-cell ca r cinom a a s well a s aden oid, trich oepitheliom atous, a n d d u cta l differentiation. H a m a rto m a to u s ba sal-cell ca r cinom a is a rare variant o f ordinary ba sal-cell car cinom a as well a s aden oid, trich o ep ith elio m atou s, a n d viduals. It is a less a gg ressive n e o p la sm a n d m a y rem ain stationary f o r ye a rs. Its recognition a s an en tity brings aw a ren ess th a t b asa l-cell carcin om as so m e tim es occu r in you n g persons.
B a sa l -c e l l c a r c i n o m a , the m ost co m m o n o f skin ca n c ers, is a locally invasive neoplasm capable of con siderable d estru ctio n o f tissue locally. F e w e r than 100 cas e s o f m etastasizing basal-cell carc in o m as have been rep o rted . M ost basal-cell c a rc in o m as arise on sunex p o se d are a s in o ld er individuals. REPORT O F A CASE
A 25-year-old white w o m an w as seen for a grow th of tw o y e a rs ' duration on the right shoulder. T he lesion w as a sy m p to m a tic . E xam ination revealed a m ass, 1.5 cm in d ia m e te r, unch anged in skin color, and coursed with telan g iectases, on the right sc ap u la r area. It was not ulcerated . Axillary and su p raclavicular nodes were not palpable. T he patient denied o v ere x p o su re to sun. T h ere w as no history o f ingestion o f arsenic. T h e fam ily history was negative for predisposition to skin can cer. Biopsy o f th e lesion revealed a neoplasm arising from the epiderm is and a follicular structure invading the derm is (Fig. 1). It w as co m posed o f islands o f small From the Departm ent o f Derm atology. Santa Monica Medical Center, Santa Monica, California (Drs. Hamilton and Zeitlin): the Departm ent o f Derm atology, Sepulveda V.A. Hospital (Dr. Hamil ton) and the Departm ent o f Dermatology. U .C .L .A . Center for Health Sciences, Los Angeles, California (Dr. Hamilton). Address reprint requests to Dr. Hamilton, W arner Victory Medi cal C enter, 6325 Topanga Canyon Boulevard, Woodland Hills, California 91367.
526
J. Derm atol. Surg. Oncol. 4 :7 July I97S
basophilic cells with focal peripheral palisading an d retraction o f s urrounding collagen charac te ristic o f basal-cell c arcinom a. A dditionally, there was follicular m alform ation show ing keratin-filled a re a s with in te r connecting s tra n d s o f basophilic cells b etw een th e is lands (Fig. 2). T h e re also was p seudoadenoid sim u la tion and duct-like stru c tu re resulting from m ucinous degeneration o f the conn e c tiv e tissue s tro m a (Fig. 3). A m o d e ra te round-cell inflam m atory infiltrate was present.
FIG U R E 1. Hamartomatous basal-cell car cinoma arising from epidermis and differentiation to follicular struc ture.
H A M IL T O N A N D Z E IT L 1 N
FIG U R E 2. Keratin-filled areas within strands o f basophilic islands o f hamurtomutous basal-cell car cinoma.
CO M M E N T
Basal-cell c a rc in o m a s are generally believed to arise from the basal germ inative lay er o f the epiderm is. T h e re fo re , as n o ted by Reidbord et a l . 1 it is not su rpris ing that cells o f v a rian t form s o f basal-cell carcinom a may exhibit ch a ra c te ristics of ad nexal stru c tu re s (i.e., hair follicles an d sw e a t ducts), since th ese elem ents arise em bryologically from these sam e basal layers. T h e h a m a rto m a to u s basal-cell c arcinom a is an e x a m ple o f maldifferentiation o f epiderm al germ inative cells. T h is variant a p p e a rs to have distinct c h a ra c te ris tics w hich delineate it from o th e r typ e s o f basal-cell ca rc in o m a s both clinically and histologically. A review o f the literature to date reveals scanty delineation of the n eo p la sm in point. Clinically, the h a m arto m a to u s basal-cell carcinom a usually h a s a history o f long end u ran ce (m ore than 30 years in one case). It begins as early as the teens, b e c o m e s w ell-d em arcated on the skin su rface, and ch aracte ristic a lly d o es not u lc erate .2 T he usual b a s a l cell c a rc in o m a , on the o th e r h an d , generally has a m uch s h o rte r h isto ry , begins in middle age o r later, is less w e ll-d e m a rc a te d , a n d characteristically ulcerates fairly early in its c o u rse . T he gross a p p e a ra n c e of h a m a rto m a to u s basal-cell carcinom as may vary; some are similar to sy rin g o m a s.2 T he lesion in o u r patient w as a largish m ass w ith telangiectasia but no ulc e ra tion. It seem s th a t actinic dam age to the skin plays little o r no role in c a u sa tio n o f these lesions. In short, early a p p e a ra n c e and long e n d u ra n c e unchanged are the m o st ch a ra c te ristic clinical featu res o f the h am a r to m a to u s basal-cell c a rcinom a.
FIG U R E 3. Pseudoadenoid simulation in hamurtomutous basal-cell car cinoma .
Histologically, a h a m a rto m a to u s basal-cell car cinom a show s differentiation to w a rd adnexal stru c tures (hair follicles, se b a c e o u s glands and eccrine ducts). It a p p e a rs that the m ost c o m m on feature seen is the keratin (horn) cyst. In o u r patient, simulation of hair follicles in the form o f keratin cy sts, glands and ducts w ere all seen. An inflammatory infiltrate m ay or may not be a tte n d a n t and it appe a rs that this feature is generally less p ronounced than in classic basal-cell c arcinom as. Calcification and mummification (shadow cells) m ay occasionally be found in the h a m a rto m a to u s basal-cell carcinom a. It has been said4 th a t the pathogenesis o f basal-cell c arcinom a is b est explained in term s o f the inability o f neoplastic epiderm al cells to form keratin. This hy p o th e sis may be valid for o rdinary basal-cell c a r cinom as, but is not as cogent for h a m a rto m a to u s basal-cell c a rcinom as b ecause considerable keratin production is c h a racteristic o f the latter.
REFERENCES 1.
2.
3.
4.
Reidbord, H. E., Wechsler, H. L ., and Fisher, E. R. Ultrastructural study o f basal cell carcinom a and its variants with com ments on histogenesis. Arch. Dermatol. 104:132-140, 1971. Schellander, F., M arks, R., and Wilson Jones, E. Basal cell ham artom a and cellular naevus: an unusual combined malfor mation. Br. J. Dermatol. 90:413-419, 1974. Johnson, W. C ., and H ookerm an. B. J. Basal cell ham artom a with follicular differentiation. Arch. Dermatol. 105:105-106, 1972. Van Scott, E. Basal cell carcinom a. In: Dermatology in General Medicine. Fitzpatrick, T., Arndt, K ., Clark, W., Eisen, A., and Van Scott, F... New York, McGraw-Hill, 1971, pp. 466-467.
./. Dermatol. Surg. Oncol. 4:7 July 1978
527
A 25-year-old w om an d e v e lo p e d a b asa l-eell carcin om a In the skin o ve r th e right sca p u la . The liistop ath olog y o f the lesion r e v e a le d elem en ts o f ty p ica l b asa l-cell ca r cinom a a s well a s aden oid, trich oepitheliom atous, a n d d u cta l differentiation. H a m a rto m a to u s ba sal-cell ca r cinom a is a rare variant o f ordinary ba sal-cell car cinom a as well a s aden oid, trich o ep ith elio m atou s, a n d viduals. It is a less a gg ressive n e o p la sm a n d m a y rem ain stationary f o r ye a rs. Its recognition a s an en tity brings aw a ren ess th a t b asa l-cell carcin om as so m e tim es occu r in you n g persons.
B a sa l -c e l l c a r c i n o m a , the m ost co m m o n o f skin ca n c ers, is a locally invasive neoplasm capable of con siderable d estru ctio n o f tissue locally. F e w e r than 100 cas e s o f m etastasizing basal-cell carc in o m as have been rep o rted . M ost basal-cell c a rc in o m as arise on sunex p o se d are a s in o ld er individuals. REPORT O F A CASE
A 25-year-old white w o m an w as seen for a grow th of tw o y e a rs ' duration on the right shoulder. T he lesion w as a sy m p to m a tic . E xam ination revealed a m ass, 1.5 cm in d ia m e te r, unch anged in skin color, and coursed with telan g iectases, on the right sc ap u la r area. It was not ulcerated . Axillary and su p raclavicular nodes were not palpable. T he patient denied o v ere x p o su re to sun. T h ere w as no history o f ingestion o f arsenic. T h e fam ily history was negative for predisposition to skin can cer. Biopsy o f th e lesion revealed a neoplasm arising from the epiderm is and a follicular structure invading the derm is (Fig. 1). It w as co m posed o f islands o f small From the Departm ent o f Derm atology. Santa Monica Medical Center, Santa Monica, California (Drs. Hamilton and Zeitlin): the Departm ent o f Derm atology, Sepulveda V.A. Hospital (Dr. Hamil ton) and the Departm ent o f Dermatology. U .C .L .A . Center for Health Sciences, Los Angeles, California (Dr. Hamilton). Address reprint requests to Dr. Hamilton, W arner Victory Medi cal C enter, 6325 Topanga Canyon Boulevard, Woodland Hills, California 91367.
526
J. Derm atol. Surg. Oncol. 4 :7 July I97S
basophilic cells with focal peripheral palisading an d retraction o f s urrounding collagen charac te ristic o f basal-cell c arcinom a. A dditionally, there was follicular m alform ation show ing keratin-filled a re a s with in te r connecting s tra n d s o f basophilic cells b etw een th e is lands (Fig. 2). T h e re also was p seudoadenoid sim u la tion and duct-like stru c tu re resulting from m ucinous degeneration o f the conn e c tiv e tissue s tro m a (Fig. 3). A m o d e ra te round-cell inflam m atory infiltrate was present.
FIG U R E 1. Hamartomatous basal-cell car cinoma arising from epidermis and differentiation to follicular struc ture.
H A M IL T O N A N D Z E IT L 1 N
FIG U R E 2. Keratin-filled areas within strands o f basophilic islands o f hamurtomutous basal-cell car cinoma.
CO M M E N T
Basal-cell c a rc in o m a s are generally believed to arise from the basal germ inative lay er o f the epiderm is. T h e re fo re , as n o ted by Reidbord et a l . 1 it is not su rpris ing that cells o f v a rian t form s o f basal-cell carcinom a may exhibit ch a ra c te ristics of ad nexal stru c tu re s (i.e., hair follicles an d sw e a t ducts), since th ese elem ents arise em bryologically from these sam e basal layers. T h e h a m a rto m a to u s basal-cell c arcinom a is an e x a m ple o f maldifferentiation o f epiderm al germ inative cells. T h is variant a p p e a rs to have distinct c h a ra c te ris tics w hich delineate it from o th e r typ e s o f basal-cell ca rc in o m a s both clinically and histologically. A review o f the literature to date reveals scanty delineation of the n eo p la sm in point. Clinically, the h a m arto m a to u s basal-cell carcinom a usually h a s a history o f long end u ran ce (m ore than 30 years in one case). It begins as early as the teens, b e c o m e s w ell-d em arcated on the skin su rface, and ch aracte ristic a lly d o es not u lc erate .2 T he usual b a s a l cell c a rc in o m a , on the o th e r h an d , generally has a m uch s h o rte r h isto ry , begins in middle age o r later, is less w e ll-d e m a rc a te d , a n d characteristically ulcerates fairly early in its c o u rse . T he gross a p p e a ra n c e of h a m a rto m a to u s basal-cell carcinom as may vary; some are similar to sy rin g o m a s.2 T he lesion in o u r patient w as a largish m ass w ith telangiectasia but no ulc e ra tion. It seem s th a t actinic dam age to the skin plays little o r no role in c a u sa tio n o f these lesions. In short, early a p p e a ra n c e and long e n d u ra n c e unchanged are the m o st ch a ra c te ristic clinical featu res o f the h am a r to m a to u s basal-cell c a rcinom a.
FIG U R E 3. Pseudoadenoid simulation in hamurtomutous basal-cell car cinoma .
Histologically, a h a m a rto m a to u s basal-cell car cinom a show s differentiation to w a rd adnexal stru c tures (hair follicles, se b a c e o u s glands and eccrine ducts). It a p p e a rs that the m ost c o m m on feature seen is the keratin (horn) cyst. In o u r patient, simulation of hair follicles in the form o f keratin cy sts, glands and ducts w ere all seen. An inflammatory infiltrate m ay or may not be a tte n d a n t and it appe a rs that this feature is generally less p ronounced than in classic basal-cell c arcinom as. Calcification and mummification (shadow cells) m ay occasionally be found in the h a m a rto m a to u s basal-cell carcinom a. It has been said4 th a t the pathogenesis o f basal-cell c arcinom a is b est explained in term s o f the inability o f neoplastic epiderm al cells to form keratin. This hy p o th e sis may be valid for o rdinary basal-cell c a r cinom as, but is not as cogent for h a m a rto m a to u s basal-cell c a rcinom as b ecause considerable keratin production is c h a racteristic o f the latter.
REFERENCES 1.
2.
3.
4.
Reidbord, H. E., Wechsler, H. L ., and Fisher, E. R. Ultrastructural study o f basal cell carcinom a and its variants with com ments on histogenesis. Arch. Dermatol. 104:132-140, 1971. Schellander, F., M arks, R., and Wilson Jones, E. Basal cell ham artom a and cellular naevus: an unusual combined malfor mation. Br. J. Dermatol. 90:413-419, 1974. Johnson, W. C ., and H ookerm an. B. J. Basal cell ham artom a with follicular differentiation. Arch. Dermatol. 105:105-106, 1972. Van Scott, E. Basal cell carcinom a. In: Dermatology in General Medicine. Fitzpatrick, T., Arndt, K ., Clark, W., Eisen, A., and Van Scott, F... New York, McGraw-Hill, 1971, pp. 466-467.
./. Dermatol. Surg. Oncol. 4:7 July 1978
527
