Case of a 13-year Old Child

The overall incidence of tumors with vulvar location is between two and seven cases per 100,000 women, and it increases with age. The mortality rate from this condition is estimated at 0.7 per 100,000 women.1

Vulvar malignancy is a rare gynecologic malignancy. Majority of the cases are squamous cell carcinomas. Adenocarcinomas are much less common. Mucinous ade-nocarcinoma may be classified into subtypes which include intestinal type, mammary gland-like adenocarcinoma and sweat gland carcinomas. 2

The clinical presentation, histopathologic studies, and management of vulvar mucinous adenocarcinoma has not been well established owing to the rarity of this type of tumor.

Immunohistochemical studies of this tumor will aid in the identification of the subtypes. Multidisciplinary approach involves surgical removal of the mass with or without chemotherapy.

CASE REPORT

This is a case of a 13-year old child, who was referred to our institution due to an enlarging mass at the right vulva.

Seven months prior to admission, the patient noted a right vulvar mass, solid, non-tender, which is gradually enlarging, from a 3x3cm mass to a 20x15cm size in ap-proximate (see Appendix, Figure 1).

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There was no associated fever, pain or difficulty in ambulation, weight loss, or change in urinary or bowel habits. No medications were taken.

One month prior to admission, the patient noticed an increase in the size of the mass, extending to her inguinal area, which was associated with pelvic pain, aggravated by walking and long hours of standing. She tolerated the condition, and took herbal medications provided by her parents.

Three days prior to admission, the mass increased in size, extending up to her inguinal area, now associated with difficulty in ambulation.

Patient sought consult in a local hospital. Examination revealed pertinent findings of right vulvar mass probably a vulvar abscess. She was admitted and given intravenous antibiotics. Pelvic ultrasound showed complex focus with mixed cystic and heterogenous echoes suggestive of pubic abscess. She was scheduled for incision and drainage under spinal anesthesia, however, during the procedure, the physician was able to aspirate suspicious tissues admixed with serous discharge. With suspicion of malignancy, the procedure was abandoned and the patient was then transferred to our institution for further evaluation and management.

The patient's past medical history was unremarkable. She is the second child of the family, with 3 siblings. She is currently a first year high school student. Family history was unremarkable, with no history of malignancy or consanguinity. Patient had no menarche yet.

On admission, she was examined conscious, coherent, with vital signs within the normal range. On physical examination, her breasts were staged as Tanner Stage 2. Focusing on the pelvic area, there was a large erythematous vulvar mass approximately 30x15cm, extending from the vulvar area up to the right inguinal area. It was warm and tender on palpation, and was noted to be tense at the lower borders. A 0.5cm movable contender lymph node was appreciated on the left inguinal area.

Intravenous clindamycin with computed dose of 10mKd every 6hours was start-ed. Pelvic Xray showed large soft tissue density at the pelvic inlet measuring 10x10cm superior displacing the bowel segments suggesting of large pelvic mass. Tumor mark-ers were taken such as lactate dehydrogenase, alpha fetoprotein, and beta-human chorionic gonadotropin which results were all normal. Chest X-ray revealed bilateral nodular densities with consideration of pulmonary metastases..

The patient was referred to pediatrics, pediatric oncology, gynecologic oncology, and pediatric surgery section for multidisciplinary management. MRI of pelvis revealed soft tissue mass measuring 14.2 x 11.4 x 10.7cm, right inguinal region, with contrala-teral lymphadenopathy (see Appendix, Figure 2). Excision biopsy was done wherein intraoperatively, a 1x1x1 tissue specimen was taken on the vulvar mass and the left inguinal lymph node for frozen tissue biopsy, which revealed rhabdomyosarcoma without lymphovascular involvement (see appendix, Figure 3).

Patient was started on first cycle of chemotherapy one week post operatively be-cause there were electrolyte imbalance that has to be corrected prior to initiating treat-ment with chemotherapy. Chemotherapy for any type of rhabdomyosarcoma is manda-tory. The agents given to the patient were Vincristine 1.8mg IVTT once dose on day 1, and Cyclophosphomide 1.45g one dose on day 1, and Actinomycin 0.5mg IVTT once a day for 5 consecutive days from day 1 to day 5 of cycle. Daily monitoring of the tumor size showed no difference from day 1 to day 7 of chemotherapy.

During the second cycle of chemotherapy, permanent section biopsy of the ex-cised mass showed mucinous adenocarcinoma, with no lymphovascular invasion and no tumor metastasis on all nine inguinal lymph nodes. At this time, chemotherapy fol-lowed by surgical excision of the mass was planned with weekly monitoring of tumor size to assess efficacy of chemotherappy. This patient was advised for further work up and to undergo immunohistochemical studies to confirm the origin and type of the tu-mor. Unfortunately, the patient's family refused further treatment, and she was subse-quently lost to follow up.

CASE DISCUSSION:

Vulvar diseases in children usually are conditions that involves a break in the skin integrity. These include contact dermatitis, injuries from trauma or sexual abuse, or infectious disorder and other extremely rare vulvar tumors. There are few reported cas-es on vulvar masses in the pediatric population. Its diagnosis is made clinically by thor-ough history and physical exmaination, supported by laboratory work-up and imaging studies, and confirmed by tissue histopathologic studies. Treatment protocols primarily address local symptoms, such as irritation, pelvic pain and difficulties in ambulation due to its location and size. Owing to the rarity of these vulvar tumors, treatment guidelines have not yet been established.

In this case, we are presented with a vulvar mass in a prepubertal patient. Infec-tious disorder was initially considered. Vulvar abscess is an accumulation of infectious fluid beneath the skin or in the subcutaneous tissue. Incision and drainage of the ab-scess usually alleviates vulvar pain, which is the common presenting symptom. Diag-nostic culture to identify specific organisms will aid in choosing appropriate antibiotic treatment. Vulvar tuberculosis is a rare condition and is seen only in 1-2% of genital tract tuberculosis. This commonly presents with chronic painful ulcerative vulvar mass associated with lymphadenopathy. In a case report of Tejinder Kaur, Surbhi Dhawan, Arti Aggarwal et. at. they presented a case of a 22 year old, complaining of ulcerative lesions in the vulva associated with difficulty in walking. Histopathologic studies showed granuloma comprising of cluster of epitheloid cells with Langhans type giant cells suggestive of tuberculosis. Anti-tuberculosis medications were started, with normal vulvar anatomy was restored after nine months of treatment.3 Vulvar tuberculosis is less likely in this case, since our patient presents with rapidly growing vulvar mass with, in contrast with genital tuberculosis. Absence of personal history of primary tuberculosis or exposure to such, also makes this diagnosis less likely.

Imaging studies done on this patient included magnetic resonance imaging, which revealed a large, well defined complex vulvar mass. Vulvar tumors were put into consideration, and these include lipoma and fibroma which are common benign tumors. Malignant tumors comprise the third category of differential diagnosis and include rhabdomyosarcoma, as well as rare epithelial cancers of the vulva, including squamous cell carcinoma and adenocarcinoma.

Being presented with a complex mass in the vulvar region in a pediatric patient, our primary considerations were vulvar fibroma and lipoma. Lipoma being the most common benign tumors of soft tissues, a tumor of mature adipocytes. However, there were only few cases reported of vulvar lipoma. One case report of Jung Hoon Lee and Seung Moon Chung, a case of a 17year old, complaining of a soft, movable mass in the right labia majora, gradually enlarging in just one year. Tumorectomy with complete removal of the capsule was done.4 Vulvar fibroma is the most common benign tumor in women. Commonly presents as a slow growing huge mass with isolated ulcerations and bleeding secondary to trauma. Tissue biopsy confirms its diagnosis. Treatment includes surgery with or without chemotherapy depending on the clinical status of the patient.5

In this case, total excision of the tumor was not done owing to its large size, as this procedure will entail removal of a large portion of the vulva which may not be ac-ceptable in this age group. Excision biopsy with frozen section biopsy was done which revealed histologic findings of rhabdomyoscaroma. The patient was started on chemo-therapy with Vincristine Cyclophosphomide and Actinomycin. These drugs were given with the goal to improve the survival and treatment of children with rhabdomyosar-coma.6

Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in pediatric population, more commonly in children below 15years old. It originates from skeletal muscles and can affect any part of the body. During childhood, a genital location for this tumor is second most common, following the head and neck. Risk factor includes familial history of rhabdomyosarcoma. This clinically presents as a tumor mass, associated with vaginal bleeding. Its advance stage, often presents with urinary symptoms. The typical histologic findings of diffuse proliferation of rhabdoid cells that are round or polygonal in shape with eccentric nuclei, prominent nucleoli, and glassy eosinophilic cytoplasm containing hyaline-like inclusion bodies is diagnostic of rhabdomyosarcoma. Immunohistochemical expression of desmin confirms the diagnosis. Multimodality therapy is the optimal therapeutic approach. Conservative therapy with chemotherapy or radiotherapy after the initial excision has been reported with excellent results. Chemotherapy is mandatory, agents used include vincristine and actinomycin with either cyclophosphamide (VAC) or ifosfamide (IVA). Prognosis if diagnosed in early stage, is very good, with an average 5-year survival of 85-90%. 1

Primary vulva cancer is a rare gynecologic malignancy and represents 3% to 5% of malignancies of the female genital tract. About 90% of primary vulva malignancies are squamous cell carcinomas and adenocarcinomas are much less common.7 Mucin-ous adenocarcinoma have different subtypes which include intestinal type, mammary gland-like adenocarcinoma and sweat gland carcinoma.2

The intestinal type or otherwise known as primary villoglandular mucinous ade-nocarcinoma of the vulva, otherwise known as cloacogenic adenocarcinoma. Hypothe-sis that intestinal neoplasia of the vulva could develop from cloacal remnants. Since the lower vagina is derived from the urogenital tract, enteric neoplasia of the lower genital tract could develop from loci of gastrointestinal metaplasia from cloacal remnants. A case of an 68-year-old woman who had a vulvar lump, with histopathologic findings of mucinous adenocarcinoma, no invasion nor microinvasion of the tumor cells was reported. This patient was then treated by vulvar tumor resection and remains disease free 5 years after surgery.8

Specialized anogenital mammary-like glands, found typically in the interlabial sulcus, are thought to be the origin of vulvar breast-like adenocarcinomas.9 Exact ma-lignant potential of ectopic breast tissue within the vulva is not known. There were 11 cases reported diagnosed with this condition, confirmed with immunohistochemical test, all of them tested for estrogen/progesterone receptors revealed positive results. Given the rarity of this entity, the incidence, clinical manifestations, and the optimal therapy and prognosis of this tumor remains unclear. 8

To identify the specific type of these tumor, extensive workup should be done, including chest X?ray, abdominal and pelvic ultrasound, cervical Pap smear and systemic positron emission tomography-computed tomography (PET-CT) to identify primary reproductive or metastatic malignancy. A colonoscopy or gastroscopy is used to exclude adenocarcinoma of the gastrointestinal tract. Immunohistochemical analysis include periodic acid?Schiff (PAS), alcian blue (AB), cyto- keratin (CK) 7 and cellular adhesion molecule (CAM) 5.2, and p16 are seen positive for primary mucinous adeno-carcinoma of the vulvar. Tumor cells positive for estrogen receptor (ER), progesterone receptor (PR) and gross cystic disease fluid protein (GCDFP) 15, are indicative of mammary gland adenocarcinoma. The tumor positive for carbohydrate antigen (CA) 125 suggests an ovarian type of cancer. Tumor stained positive for CK20, villin and CA19-9 suggest adenocarcinoma of the gastrointestinal tract. The tumor stained posi-tive for transcription intermediary factor (TTF) 1 and napsin A is indicative of lung adenocarcinoma. Thus, to further specify these type of tumors, immunohistochemical studies is a valuable tool. 2

Vulvar biopsies are of diagnostic important, wherein a sample from the mass is excised for histopathologic studies. Diagnostic biopsies may be punch biopsy to evalu-ate inflammatory process or an excisional biopsy, preferred over punch biopsy, for sus-pected neoplastic tumors. 10

There are several approach in the management of this malignancy. Patients with a locally advanced primary or nodal disease should be referred for multidisciplinary clinical evaluation.12 The overall goal is to remove all involved tissue affected by the vulvar cancer, including the skin from the vulva to the inguinal area.11 A radical local excision or a total vulvectomy are the surgical options, with or without lymph node dissection. According to the clinical practice guidelines from Alberta Provincial Gynecologic Oncology Team, for patients who are not candidates for surgery, an aprp radiation therapy or chemoradiotherapy could be considered for primary definitive treatment, as an alternative to surgery. However, salvage surgery should be considered if no response is documented after three months. For advanced vulvar cancer, neoadjuvant radiation with or without chemotherapy followed by radical surgery should be considered the best option for management.12

Prognosis after surgical procedure followed with chemotherapy showed promis-ing results. The 5-year survival rate of all types of vulval cancer is 96.0% for individuals aged 20-60 years and 68.8% for individuals aged 70 years and older. 12

SUMMARY:

Most vulvar mass are diagnosed in adult women, and rare cases in pediatric age group. With my readings, this is the first case of vulvar mucinous adenocarcinoma in this age group.

Vulvar mucinous adenocarcinoma is a rare malignant condition with promising prognosis if diagnosed at less advanced stage. Its complexity requires a series of labor-atory test and histopathologic studies. There are several surgical approach with the aim to totally remove the malignant tissues and prevent recurrence.

Cancer is a debilitating condition that changes not just our patients' life but also their family as well. It is our role as a physician to thoroughly discussed with the patient together with her family, the extent of the condition, the management and its possible outcome. Currently, we are still looking for ways to bring this patient back to treatment. We are trying to talk to the parents and convince them to agree with further treatment.