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1233
Case Report
Familial Mark
Splenic
W. Ragozzino,1
Henry
Epidermoid Singletary,2
and
Cyst
Ronald
Patrick3
Abdominal CT showed a complex splenic cyst and free intrapentoneal fluid or hemorrhage. Splenectomy was performed. There was 1300 ml of gray-green intrapentoneal fluid. The spleen weighed 1 1 50 g and
The differential diagnosis of cystic lesions of the spleen includes cystic neoplasms (sarcoma, lymphangioma, hamartoma), parasitic cysts (echinococcosis), nonparasitic abscess, pseudocysts (without an epithelial lining), and epithelial cysts [1 -3]. Epithelial cysts can be classified as mesothelial, epidenmoid, and dermoid. Epidermoid cysts are now generally believed to be due to squamous metaplasia within preexisting mesothelial cysts [2, 4]. Dermoid cysts possess skin adnexa and squamous epithelium. Very few have been reported, and their existence is controversial [5]. We observed three siblings with splenic epidermoid cysts. Splenic rupture occurred in two of the three siblings. The familial occurrence of epider-
measured
20 x 10 x 1 0 cm. Cultures of the intraperitoneal fluid were Gross section of the spleen contained three cysts measur4.0, and 3.0 cm, respectively. The three cysts were lined
negative. ing
1 4.5,
almost entirely by stratified beculated appearance.
squamous
epithelium
and
had
a tra-
The 21 -year-old sister of the index patient was brought to the emergency department. She complained of persistent, dull, upper abdominal pain with radiation to the back; nausea; and weight during the past year. Results of esophagogastroduodenoscopy
loss
this case report.
6 months earlier had been normal. Sonography showed a 3.5-cm simple cyst and a separate 4.5-cm cyst with low-level echoes compatible with either hemorrhage or infection (Fig. 1 A). A follow-up CT scan
Case
frequent
backpacking
prompted
prophylactic
moid cysts and their apparent
tendency
to rupture
prompted
obtained 3 days later (Fig. 1 B) confirmed the sonographic The patient’s persistent symptoms, active life-style (which
Reports
A 16-year-old boy collided with another player while playing baseball and later developed pain and fullness in the left upper quadrant. Abdominal CT showed a large splenic cyst and a large amount of intraperitoneal fluid or hemorrhage. Splenectomy was performed. There was 2000 ml of old intrapentoneal 1 2 x 9 cm unilocular containing hemorrhagic
trips to remote splenectomy.
revealed two splenic epidermoid Review
uncles,
of the health
cousins,
cyst lined by firm, material. Microscopic
analysis
parents,
aunts,
no other
splenic
Epithelial cysts of the spleen are uncommon, making up approximately 1 0% of benign, nonparasitic cysts [6], as determined on the basis of referral practices. Approximately 200 cases of splenic epidermoid cysts have been described in the
English literature.
The mean age of patients
epidermoid
is approximately
cysts
1
February 22, 1990; accepted after revision May 20, 1990. Delaney Radiologists, 2212 Delaney Ave., Wilmington, NC 28401 . Address reprints requests to M. W. Ragozzino.
2
Wilmington
3
Department
1626 Harbour
Dr., Wilmington,
NC 28402.
of Surgery, New Hanover Memorial Hospital, Wilmington, December
revealed
Discussion
of the cyst
Received
AJR 155:1233-1234,
grandparents,
and nieces
abnormalities.
The 23-year-old pregnant sister of the index patient was incidentally observed to have a large splenic cyst on screening obstetric sonography. She came to the emergency department 3 weeks postpartum complaining of pain in the back and left upper quadrant.
Associates,
history analysis
blood. There was an 1 1 x white, trabeculated tissue
revealed an inner lining of stratified squamous epithelial cells with some areas of nonciliated, nonsecretory cuboidal (mesothelial) cells. The cyst wall was partially calcified.
Pathology
and familial
and microscopic
cysts (Figs. 1C and 1D).
of the siblings’
nephews,
areas),
Gross
findings. included
1990 0361 -803X/90/1
556-1233
NC 28402.
© American
Roentgen Ray Society
20 years,
diagnosed with
with
a range
Downloaded from www.ajronline.org by 181.118.176.211 on 10/08/15 from IP address 181.118.176.211. Copyright ARRS. For personal use only; all rights reserved
Fig. 1.-A, Sonogram shows simple cyst of spleen. B, cr scan shows simple cyst of spleen. c, Photograph of surgical specimen shows enlarged spleen with two cysts. D, Photomicrograph of well-formed stratified squamous epithelial lining in dense connective tissue. (H and E x250)
from 6 months to 62 years [7]. No strong sexual predominance is observed. The natural history of splenic epidermoid cysts is unknown; no reports of long-term follow-up of a cohort of persons with splenic cysts have been published. Clinical presentations include painless or painful splenomegaly, spontaneous or
do not allow confident genetic mapping of this trait and elucidation of possible mechanisms. An accurate assessment of the natural history of sporadic or familial splenic epidermoid cysts remains unknown. However, given the potential for splenic rupture, noninvasive son-
posttraumatic
cysts may be warranted so that appropriate precautions and prophylaxis can be undertaken by those, albeit rare, persons found to have splenic cysts. Management depends on presentation and the life-style of the patient. Options include careful observation, partial or complete splenectomy [7, 8],
splenic
rupture,
infection,
intractable
nausea,
and dyspnea [3, 5-1 2]. Several authors describe exacerbation of the signs and symptoms during menstruation [9] and pregnancy [8, 10]. Epidermoid cysts of the spleen are now generally believed to represent metaplasia within mesothelial cysts. These are postulated to arise from invagination of the surface mesothehum during spleen formation [1 , 3, 4] or trapping of peritoneal mesothelium after rupture ofthe splenic capsule [1 , 2]. Splenic epidermoid cysts are not associated with cysts in any other organ. The overwhelming majority of cases are sporadic, as would be expected from the theories of epidermoid formation and the absence of associated abnormalities. Two case reports of familial splenic epidermoid cysts have been published [1 1 , 1 2]. Two of five siblings in one family were diagnosed with epidermoid splenic cysts [1 1 ]. Three of six siblings in another family had epidermoid cysts of the spleen [1 2]. Clinical presentations included asymptomatic splenomegaly,
dyspnea,
left shoulder
pain,
tenderness
in the
left upper quadrant, and persistent vomiting. Splenic rupture or infection was not observed in these five patients. No evidence or history of splenic disease was elicited in either of the families. Our observation of splenic epidermoid cysts in siblings and two prior case reports [11, 12] suggest an autosomal recessive, organ-specific defect of mesothelial migration in a small proportion of persons with splenic epidermoid cysts. The rarity of this observation and the lack of associated disorders
ographic
screening
marsupialization,
of
siblings
of
and percutaneous
persons
with
epidermoid
aspiration.
REFERENCES 1 . Dachman
A, Ros P, Muran
P, Olmsted
W, Lichtenstein
J. Non-parasitic
splenic cysts: a report of 52 cases with radiologic-pathologic AJR 1986;147:537-542 2. Burrig K. Epithelial (true) and so-called epidermoid 12:275-281
3. Fowler RH. Non-parasitic 4. 5.
6. 7.
8. 9. 1 0. 11. 12.
correlation.
splenic cysts: pathogenesis of the mesothelial cyst of the spleen. Am J Surg Pathol 1988;
benign cystic tumors of the spleen. mt Abstr Surg 1953;96:209-227 Ough Y, Nash H, Wood D. Mesothelial cysts of the spleen with squamous metaplasia. Am J Gun Pathol 1981;76:666-669 Pujari B, Phatah A, Jayaranariah M, Deodhare S. Dermoid cyst of the spleen. mt Surg 1976;61 :603-604 Ross M, ElIwood R, Yang S, Lucas R. Epidermoid splenic cysts. Arch Surg 1977;1 12:596-599 Park J, Song T. Splenic cyst: a case report and review of the literature. Am Surg 1971;37:544-547 Doolas A, Nolte M, McDonald 0, Economu S. Splenic cysts. J Surg Oncol 1978;10:369-387 Tamaki H. Splenic cysts. Arch Pathol 1948;46:550-557 Aiengar N. Solitary splenic cysts. Indian Med Gazette 1945;80: 131-138 Ahlgren L, Beardmore H. Solitary epidermoid splenic cysts: occurrence in sibs. J Pediatr Surg 1984;19:56-58 Gilmartin D. Familial multiple epidermoid cysts of the spleen. Conn Med
1978;42:297-300
1233
Case Report
Familial Mark
Splenic
W. Ragozzino,1
Henry
Epidermoid Singletary,2
and
Cyst
Ronald
Patrick3
Abdominal CT showed a complex splenic cyst and free intrapentoneal fluid or hemorrhage. Splenectomy was performed. There was 1300 ml of gray-green intrapentoneal fluid. The spleen weighed 1 1 50 g and
The differential diagnosis of cystic lesions of the spleen includes cystic neoplasms (sarcoma, lymphangioma, hamartoma), parasitic cysts (echinococcosis), nonparasitic abscess, pseudocysts (without an epithelial lining), and epithelial cysts [1 -3]. Epithelial cysts can be classified as mesothelial, epidenmoid, and dermoid. Epidermoid cysts are now generally believed to be due to squamous metaplasia within preexisting mesothelial cysts [2, 4]. Dermoid cysts possess skin adnexa and squamous epithelium. Very few have been reported, and their existence is controversial [5]. We observed three siblings with splenic epidermoid cysts. Splenic rupture occurred in two of the three siblings. The familial occurrence of epider-
measured
20 x 10 x 1 0 cm. Cultures of the intraperitoneal fluid were Gross section of the spleen contained three cysts measur4.0, and 3.0 cm, respectively. The three cysts were lined
negative. ing
1 4.5,
almost entirely by stratified beculated appearance.
squamous
epithelium
and
had
a tra-
The 21 -year-old sister of the index patient was brought to the emergency department. She complained of persistent, dull, upper abdominal pain with radiation to the back; nausea; and weight during the past year. Results of esophagogastroduodenoscopy
loss
this case report.
6 months earlier had been normal. Sonography showed a 3.5-cm simple cyst and a separate 4.5-cm cyst with low-level echoes compatible with either hemorrhage or infection (Fig. 1 A). A follow-up CT scan
Case
frequent
backpacking
prompted
prophylactic
moid cysts and their apparent
tendency
to rupture
prompted
obtained 3 days later (Fig. 1 B) confirmed the sonographic The patient’s persistent symptoms, active life-style (which
Reports
A 16-year-old boy collided with another player while playing baseball and later developed pain and fullness in the left upper quadrant. Abdominal CT showed a large splenic cyst and a large amount of intraperitoneal fluid or hemorrhage. Splenectomy was performed. There was 2000 ml of old intrapentoneal 1 2 x 9 cm unilocular containing hemorrhagic
trips to remote splenectomy.
revealed two splenic epidermoid Review
uncles,
of the health
cousins,
cyst lined by firm, material. Microscopic
analysis
parents,
aunts,
no other
splenic
Epithelial cysts of the spleen are uncommon, making up approximately 1 0% of benign, nonparasitic cysts [6], as determined on the basis of referral practices. Approximately 200 cases of splenic epidermoid cysts have been described in the
English literature.
The mean age of patients
epidermoid
is approximately
cysts
1
February 22, 1990; accepted after revision May 20, 1990. Delaney Radiologists, 2212 Delaney Ave., Wilmington, NC 28401 . Address reprints requests to M. W. Ragozzino.
2
Wilmington
3
Department
1626 Harbour
Dr., Wilmington,
NC 28402.
of Surgery, New Hanover Memorial Hospital, Wilmington, December
revealed
Discussion
of the cyst
Received
AJR 155:1233-1234,
grandparents,
and nieces
abnormalities.
The 23-year-old pregnant sister of the index patient was incidentally observed to have a large splenic cyst on screening obstetric sonography. She came to the emergency department 3 weeks postpartum complaining of pain in the back and left upper quadrant.
Associates,
history analysis
blood. There was an 1 1 x white, trabeculated tissue
revealed an inner lining of stratified squamous epithelial cells with some areas of nonciliated, nonsecretory cuboidal (mesothelial) cells. The cyst wall was partially calcified.
Pathology
and familial
and microscopic
cysts (Figs. 1C and 1D).
of the siblings’
nephews,
areas),
Gross
findings. included
1990 0361 -803X/90/1
556-1233
NC 28402.
© American
Roentgen Ray Society
20 years,
diagnosed with
with
a range
Downloaded from www.ajronline.org by 181.118.176.211 on 10/08/15 from IP address 181.118.176.211. Copyright ARRS. For personal use only; all rights reserved
Fig. 1.-A, Sonogram shows simple cyst of spleen. B, cr scan shows simple cyst of spleen. c, Photograph of surgical specimen shows enlarged spleen with two cysts. D, Photomicrograph of well-formed stratified squamous epithelial lining in dense connective tissue. (H and E x250)
from 6 months to 62 years [7]. No strong sexual predominance is observed. The natural history of splenic epidermoid cysts is unknown; no reports of long-term follow-up of a cohort of persons with splenic cysts have been published. Clinical presentations include painless or painful splenomegaly, spontaneous or
do not allow confident genetic mapping of this trait and elucidation of possible mechanisms. An accurate assessment of the natural history of sporadic or familial splenic epidermoid cysts remains unknown. However, given the potential for splenic rupture, noninvasive son-
posttraumatic
cysts may be warranted so that appropriate precautions and prophylaxis can be undertaken by those, albeit rare, persons found to have splenic cysts. Management depends on presentation and the life-style of the patient. Options include careful observation, partial or complete splenectomy [7, 8],
splenic
rupture,
infection,
intractable
nausea,
and dyspnea [3, 5-1 2]. Several authors describe exacerbation of the signs and symptoms during menstruation [9] and pregnancy [8, 10]. Epidermoid cysts of the spleen are now generally believed to represent metaplasia within mesothelial cysts. These are postulated to arise from invagination of the surface mesothehum during spleen formation [1 , 3, 4] or trapping of peritoneal mesothelium after rupture ofthe splenic capsule [1 , 2]. Splenic epidermoid cysts are not associated with cysts in any other organ. The overwhelming majority of cases are sporadic, as would be expected from the theories of epidermoid formation and the absence of associated abnormalities. Two case reports of familial splenic epidermoid cysts have been published [1 1 , 1 2]. Two of five siblings in one family were diagnosed with epidermoid splenic cysts [1 1 ]. Three of six siblings in another family had epidermoid cysts of the spleen [1 2]. Clinical presentations included asymptomatic splenomegaly,
dyspnea,
left shoulder
pain,
tenderness
in the
left upper quadrant, and persistent vomiting. Splenic rupture or infection was not observed in these five patients. No evidence or history of splenic disease was elicited in either of the families. Our observation of splenic epidermoid cysts in siblings and two prior case reports [11, 12] suggest an autosomal recessive, organ-specific defect of mesothelial migration in a small proportion of persons with splenic epidermoid cysts. The rarity of this observation and the lack of associated disorders
ographic
screening
marsupialization,
of
siblings
of
and percutaneous
persons
with
epidermoid
aspiration.
REFERENCES 1 . Dachman
A, Ros P, Muran
P, Olmsted
W, Lichtenstein
J. Non-parasitic
splenic cysts: a report of 52 cases with radiologic-pathologic AJR 1986;147:537-542 2. Burrig K. Epithelial (true) and so-called epidermoid 12:275-281
3. Fowler RH. Non-parasitic 4. 5.
6. 7.
8. 9. 1 0. 11. 12.
correlation.
splenic cysts: pathogenesis of the mesothelial cyst of the spleen. Am J Surg Pathol 1988;
benign cystic tumors of the spleen. mt Abstr Surg 1953;96:209-227 Ough Y, Nash H, Wood D. Mesothelial cysts of the spleen with squamous metaplasia. Am J Gun Pathol 1981;76:666-669 Pujari B, Phatah A, Jayaranariah M, Deodhare S. Dermoid cyst of the spleen. mt Surg 1976;61 :603-604 Ross M, ElIwood R, Yang S, Lucas R. Epidermoid splenic cysts. Arch Surg 1977;1 12:596-599 Park J, Song T. Splenic cyst: a case report and review of the literature. Am Surg 1971;37:544-547 Doolas A, Nolte M, McDonald 0, Economu S. Splenic cysts. J Surg Oncol 1978;10:369-387 Tamaki H. Splenic cysts. Arch Pathol 1948;46:550-557 Aiengar N. Solitary splenic cysts. Indian Med Gazette 1945;80: 131-138 Ahlgren L, Beardmore H. Solitary epidermoid splenic cysts: occurrence in sibs. J Pediatr Surg 1984;19:56-58 Gilmartin D. Familial multiple epidermoid cysts of the spleen. Conn Med
1978;42:297-300
