Clinic-Pathologic Conference

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					 視訊會議
2010-06-23

    嘉義長庚醫院
    主講: 家醫科 夏明輝醫師
    討論: 小兒外科 王世憲醫師
        小兒科 顏如貝醫師
        精神科 蔡景淑醫師
Case Report

   Name: 黃X凱
   Age: 10 m/o
   Sex: male
   Body length: 72.5CM
   Body weight: 9.5KG
   Admission: 2010-4-7
   Discharge: 2010-4-9
History

 Birth History
  G1P1, GA: 39+3 weeks, C/S
  BBW: 3075 gm
  Apgar score: 8 (1min) --> 9 (5min)
 Family History
  No history of testicular lesion
  No history of cancer
Chief Complaint

   Left scrotal enlargement noted for 10+days
Present Illness

   Left scrotal enlargement was found around 10+
    days ago while bathing. The size of left scrotum
    seemed no change during this period and there
    was no pain or irritable crying noted.
   Hernia or hydrocele was suspected at LMD (雲
    林虎尾Dr. 邢’ pediatric clinic) and was referred
    to our PS OPD for further evaluation.
   At our PS OPD: A hard, round, movable, and
    non-painful mass was palpable in left scrotal. Its
    size was around 2 cm x 2 cm.
As there was no transillumination at PS OPD,
 series workup included:
-- Sonogram: one multiseptal mass in the left
 scrotum, with size of 1.46 x 1.17 x 1.15 cm.
 No normal left testis can be demonstrated
-- Chest x-ray: no specific finding
-- Lab data showed AFP 55.14 ng/mL, BETA-
 HCG< 2.0mIU/mL
Physical Examination

   General Appearance: moderated development and moderated
    nutrition
   Activity: fair, Consciousness: clear, E4V5M6
   Skin: no rashes, no petechiae
   HEENT: negative finding, NECK: supple, no lymphadenopathy
   CHEST: symmetric expansion
              BS: clear, HS: RHB, no murmur
   ABDOMEN: soft and flat, no palpable mass, no tenderness
   Extremities: freely movable, no pitting edema
   Bilateral groin areas: no lymphadenopathy, no palpable mass
   Scrotum: Right testes in situ with normal size
                A hard and movable mass around 2.0 cm x 2.0 cm in left
                scrotum
Laboratory Data
           WBC     RBC     Hb      Plt    aFP     bHCG     LDH


Normal     5.3-    4.28-   11.6-   150-   <15     <5       98-192
range      12.0k   5.05M   13.7    400K   ng/ml   mIU/ml   U/L

99/03/30   11.6K   4.50M   12.3    383K   55.14   <2.0


99/04/16                                  40.44   <2.0     225

99/05/29   8.5K            12.1    267K   42.9    <2.0     216
Chest PA Radiogram




     99-03-30        99-05-30
Sonogram (99-03-30)




• One multiseptal mass in the left scrotum, size: 1.46 x 1.17
 x 1.15 cm, and left testis can not be demonstrated
• Right testis in the scrotum, size: 1.19 x 1.07 x 0.7 cm
 --Impression: left scrotum mass, origin from the left testis?
Pre-surgery impression

   Left testicular tumor
 -- Teratoma?
 -- York sac tumor ?
 -- Stromal tumors ?
     Sertoli’s cell tumor
     Leydig cell tumor
OP (99-4-8):Left Orchiectomy with high
ligation of spermatic cord

1.   Left scrotal approach to expose the left testicular mass
2.   A testicular mass of about 2 x 2 cm in size, with cystic like lesion on
     the surface of turnica albuginea, no normal testicular tissue found
     grossly
3.   Clamped the spermatic cord before manipulation, further
     mobilization of the spermatic cord to the level of internal inguinal
     ring
4.   Testicular tumor with york sac tumor vs teratoma was highly
     suspected and explained to his family
5.   Left orchiectomy with high ligation of spermatic cord was done (the
     spermatic cord was suture-ligated at the level of internal ring with
     its stump secured to the internal ring orifice)
Pathology
   DX:
     TESTIS, LEFT, ORCHIEDECTOMY
        ----TERATOMA (pT1 NX MX SX; STAGE I)
     SPERMATIC CORD, LEFT, RESECTION
        ----NO PATHOLOGICAL DIAGNOSIS MADE

   GROSS D:
      THE SPECIMEN SUBMITTED CONSISTS OF ONE ORCHIDECTOMY
    SPECIMEN INCLUDING SPERMATIC CROD: MEASURING 2.5 X 2.5 CM
    OF THE TESTIS AND 4.5 CM IN LENGTH OF THE SPERMATIC
    CORD. THERE IS A 2 X 2 CM ILL-DEFINED CYSTIC MASS IN THE
    TESTIS. ALL ARE SUBMITTED AND LABELED A TO F.       DON/YSL
   MICRO D:
      THE TERATOMA IS EMBEDDED WITHIN THE TESTICULAR
    PARENCHYMA AND IS COMPOSED OF MATURE COMPONENTS OF
    INTESTINAL MUCOSA, RESPIRATORY MUCOSA, CARTILAGE,
      MUSCLE, AND STRUCTURE OF 3RD VENTRICLE, AND SOME
    IMMATURE NEURAL TISSUE.
Final diagnosis

  Left Testicular Teratoma (pT1 NX MX SX; Stage I)
  Follow up
1. Physical examination and Laboratory (aFP, HCG,
   LDH) per month
2. Image: sonogram / 3 months
         Abdominal and perineal CT if necessary
3. Consult Psychologist
Discussion




    小兒外科 王世憲醫師
How to approach a child with “scrotal swelling”?
-- History


 --How long have it happened?
 --Any cause? (trauma, inset bite, abuse)
 --recently URI, AGE
 --Sudden onset vs. Gradually enlarged
 --Will it regressed spontaneously?
 -- Painless vs. Pain? Degree of severity?
 -- Urinary symptoms? Abdominal symptoms?
How to approach a child with “scrotal swelling”?
-- Physical Examination

  A gentle, reassuring manner, warm environment,
   noninvolved side examined first
 Observation:
 --A relaxed or contracted scrotum
 --Degree of swelling and erythema
 --Bruizing, Discoloration, or Blue spot?
 Palpation:
 --Cremaster muscle reflex
 --Groin bulging mass? Soft or firmly? Reducible?
 --Spermatic cord– thick? Tender?
 --Testes: size, lifting pain, local or diffuse tenderness
Other Diagnostic Studies
   Sonogram
   Color Doppler Ultrasound
   99AMP Radionuclide scanning
   MRI
Germ cell tumor (>90% of testes tumor)
    Adult
1.   Mostly: seminoma, nonseminomatous GCT
2.   Pathogenesis: [i(12p)]
3.   Aneuploid, mixed histology
4.   Malignant course
    Pediatric
1.   Mostly: yolk sac tumor, teratoma
2.   Pathogenesis: del1p, primodial germ cell migration to
     the genital ridge during development
3.   Diploid (except yolk sac tumor), pure histology
4.   Benign course
     Pediatric Testicular Tumors

1.   1 % of all pediatric solid tumors (Cancer J
     Clin 2009; 59:225)
2.   Incidence: 0.5-2.0 / 100,000 children
3.   Fundamentally different from their
     postpubertal counterpart
4.   Since 1980, Prepubertal testes tumor registry
     (PTTR) by the urological section of American
     Academy of Pediatrics epidemiological data
5.   Still no prospective randomized trials to
     delineate the best treatment
Tumor types for primary tumors in boys <12
y/o




           The Journal of Urology, 168: 1675-1679, 2002
•Children’s National Medical Center, Washington, D.C.+ Children’s Hospital of Philidelphia, Pennsylvania +
Hospital for Sick Children, Toronto, Canada + Children’s Hospital, Boston, Massachusetts
 Period=17, 25, 19, 11 yrs. N=98      The Journal of Urology, 172: 2370-2372, 2004
Is AFP a reliable tumor marker?
Reference values of serum AFP
The Journal of Urology, 168: 1675-1679, 2002
AFP level (ng./ml.) as function of age for infants with yolk sac tumor or teratoma
The Journal of Urology, 168: 1675-1679, 2002
Kaplan-Meier survival curves by stage for registry patients with yolk sac tumor and
complete survival and stage information
The Journal of Urology, 168: 1675-1679, 2002
Platinum based, multi-agent chemotherapy post
orchiectomy
 The UK children’s center study group (J Clin
  Oncol, 18: 3809, 2000)
 German Society of Pediatric Oncology (Klin
  Padiatr, 211: 300, 1999)
 The US Children’s Oncology Group (Med Ped
  ncol, 29: 413, 1997)
  Cx for Stage I recurrence or high stage
  All cured
Teratoma
   Uniformly benign in children, all cured in PTTR
   Pure histology with diploid DNA
   3 embryological germ cell layers with cartilage, fat, bone,
    muscle,neural element
   Normal serum AFP (or <100 ng/ml)
   Malignant in adult and postpubertal children
   Testes sparing surgery recommended
   Frozen section after enucleation to confirm diagnosis
    and complete resection
   Pubertal change in histology necessitate conversion to
    total orchiectomy


                           The Journal of Urology, 176: 875-881, 2006
Yolk sac tumor
(Endodermal sinus tumor)
   Stage I: 85% in children, 35% in adult
   Pure histological type in child
   AFP is a reliable marker for Dx and followup
   Hematogenous spread (20% lung meta) >
    lymphatic spread (4% retroperitoneal LAP)
   Radiacal inguinal orchiectomy
   Platinum based, multi-agent chemotherapy for
    stage II-IV post orchiectomy
   RPLND limited to persistent RPLAP or AFP↑
    post chemotherapy
                       The Journal of Urology, 176: 875-881, 2006
Algorithm for initial surgical management of prepubertal patients with testis tumor
The Journal of Urology, 168: 1675-1679, 2002
     Ultrasonogram:
differentiation diagnosis



            小兒科顏如貝醫師
Ultrasonogram (US) for testicular
tumor

   To help distinguish intratesticular from extratesticular lesions, because
    the majority of extratesticular masses are benign and intratesticular
    masses are more likely to be malignant. US does not provide the
    histologic and morphologic diagnosis.
   Gray-scale US is nearly 100% sensitive for detection of testicular
    tumors. There are a variety of benign intratesticular processes, such
    as hematoma, orchitis, abscess, infarction, and granuloma, that mimic
    testicular malignancy and must therefore be considered in the
    differential diagnosis.
   Color Doppler and power Doppler US demonstrate increased
    vascularity in the majority of malignant tumors and help to better define
    testicular involvement. The presence of hypervascularity is not specific
    enough for a diagnosis of malignancy, and it may be difficult to
    demonstrate increased blood flow in small tumors

           Sonography of the Scrotum. Radiology 2003; 227(1):18-36
Classification of testicular tumors
UpTodate: Clinical manifestations, diagnosis, and staging of testicular germ cell tumors

1. Germ cell tumors (95%)                     3. Mixed germ cell and stromal elements
Seminoma                                      Gonadoblastoma
Nonseminomatous
 .Embryonal carcinoma                         4. Adnexal and paratesticular tumors
 .Teratoma                                    Adenocarcinoma of rete testis
 .Choriocarcinoma                             Mesothelioma
 .Yolk sac tumor
 .Mixed germ cell tumor                       5. Miscellaneous tumors
                                              Carcinoid
2. Sex cord-stromal tumors                    Lymphoma
Sertoli cell tumor                            Testicular metastases
Leydig cell tumor
Granulosa cell tumor
Mixed types (eg, Sertoli-Leydig cell tumor)
       2010/6/23
罹癌(生病) 對嬰兒期幼兒與其父母的影響
        ~ 以個案為例



          精神科 蔡景淑醫師
Cognitive development

   Piaget’s model
            As the result of biological maturation and the
             accumulation of experience
    –   The sensorimotor period (infancy)
    –   The preoperational period (early childhood,
        roughly 2 to 7 years)
    –   The concrete operational period (middle to late
        childhood, roughly 7 to 11 years)
    –   The formal operational period (adolescence to
        adulthood)
            Abstract thought attained
Piaget’s sensorimotor period of
cognitive development

   Birth – 2 mos
    –   Uses inborn motor and sensory reflexes (sucking, grasping,
        looking) to interact and accommodate to the external world
   2-5 mos
    –   Primary circular reaction: coordinates activities of own body
        and five senses (e.g. sucking thumb); reality remains
        subjective - doesn’t seek stimuli outside of its visual field;
        displays curiosity
   5-9 mos
    –   Secondary circular reaction: seeks out new stimuli in the
        environment; starts both to anticipate consequences of own
        behavior and to act purposely to change the environment;
        beginning of intentional behavior
Piaget’s sensorimotor period of
cognitive development

   9 mos – 1 yr
    –   Shows preliminary signs of object permanence; have a
        vague concept that objects exist apart from itself; plays
        peek-a-boo; imitates novel behaviors
   1 yr – 18 mos
    –   Tertiary circular reaction: seeks out new experiences;
        produces novel behavior
   18 mos – 2 yrs
    –   Symbolic thought: uses symbolic representations of events
        and objects; showing signs of reasoning (e.g. uses one toy
        to reach for and get another); attains object permanence
住院的影響—嬰兒期與學齡前期

   出生到六個月大
    – 親情固著前期   (尚未對照顧者有固定化的親情)
        對照顧者的改變仍有所反應
    – 住院後可觀察到的行為
        雖沒有很明顯的不適,但總是顯得與新環境格格不入,一
         直在察言觀色,警覺新環境
        出現餵食與睡眠的障礙,眼睛失去注視與傳情的能力
    – 在這個階段的嬰孩,住院會剝奪一些有利發展的社
     會文化刺激,如抱、輕搖、愛撫、遊玩與談話等等
     的人際互動關係
        不舒服、痛苦、被約束、被限制活動…
住院的影響—嬰兒期與學齡前期

   六個月到一歲
    –   已達到親情固定化的階段
           感情牽繫主要在照顧者身上
    – “物體恆存”概念還未成熟
           如:看不到照顧者就以為照顧者消失不見了
    – 住院後可觀察到的行為
           焦慮不安,默默無言,怕陌生人,餵食困難,腸胃不適,
            一回到家就有分離焦慮
    – 住院會剝奪一些人際的互動關係,限制了孩子的活
        動,影響到孩子的發展,但只是短暫的影響
 For this case ( 11 mos )
                  原始分數 發展年齡(月)   歲       月    DQ (%)
粗動作    GM / 34     11    10      0   :   10     91
精細動作   FM / 44     12     8      0   :   8      73     *
溝通表達   EL / 54     8      8      0   :   8      73     *

概念理解   CC / 67     3     10      0   :   10     91

環境理解   SC / 44     7      7      0   :   7      64     **

身邊處理   SH / 36     3      8      0   :   8      73     *

人際社會   PS / 34     7     10      0   :   10     91

一般發展   GD / 138    19    10      0   :   10     91
                                      個案發展情形

    For this case ( 11 mos )
         學齡前兒童行為發展量表簡圖
                                      個案生理年齡
                                      delay 20%
                                      delay 30%

    84
    78
    72
    66
發
    60
展   54
年   48
齡   42
    36
︵
    30
月   24
︶   18
    12
    6
    0

         粗    精    溝    概    環    身       人        一
         動   作細   達通   解念   解境   理邊      會際       展般
         作    動    表    理    理    處       社        發
Adjustment and resilience by
family of children with cancer
Situation of adjustment

   2/3 parents at lowest risk
   1/3 at elevated risk
   Rarely in high risk
   Parents of relapsed children
    –   At risk for long-term psychological problems
    –   High level of psychosomatic complaints
Situation of adjustment

   Difference evidences in level of emotional
    disturbance to anxiety/depression
   Different level of sleep disturbances to
    insomnia
   Emotional consequences decline over time
Parental adjustment

●Children with cancer surviving,
  parents would …
 Fear for relapses
 Self-blame
 Worries about infertility
 Feelings of uncertainty
 Uncertainties about future
Parental adjustment

●Children with cancer surviving,
  parents would …
 Find other support systems
 Re-evaluations of their goals and life
Parental adjustment

●Marital crisis?
 No higher divorce rate
  no significance than normal group
 Increasing studies found marital distress or
  sexual relationship
Social support

   Quality of parent-staff relationship was best
    predicted positive personal contact
   Studies report that family and friends was
    very helpful
   Similar parents with cancer child would be
    most helpful
   Mothers appear to use social support more
    than father
For the parents of this case
~ Parental adjustment

   Fear for relapses
   Self-blame
   Worries about infertility
   Feelings of uncertainty
   Uncertainties about future
   Find other support systems
   Re-evaluations of their goals and life
For the parents of this case
~ Parental adjustment

Fear for relapses
 Worries about infertility
 Self-blame
→give education about disease
→support family
→strength family resilience
For the parents of this case
~ Social support

   Parent-staff relationship
   Family and friends
   Similar parents
Childhood cancer survivors

   A group at increased risk for maladaptive
    psychosocial squalae, behavioral adjustment
    problems and anxiety
    –   Conflicting findings
   Severity of disease and intensity of treatment is
    related to the ability to cope with the illness
    experience

				
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